,aj\/%y 


^%/ 


/' 


,"V?f%^XA^'i 


RC^4l 


J^9 


mtljeCttpaflifttsgork 

of  ^Jii^iStciang  anb  ^urgeonss 
iCibrarp 


Br.  M:   A.StARR 


Digitized  by  the  Internet  Archive 

in  2010  with  funding  from 

Open  Knowledge  Commons 


http://www.archive.org/details/diseasesofnervoOOjell 


DISEASES 


NERVOUS  SYSTEM 


A  TEXT-BOOK  OF 


NEUROLOGY  AND  PSYCHIATRY 


BY 

SMITH  ELY  JELLIFFE,  M.D.,  Ph.D. 

ADJUNCT    PROFESSOR    OF    DISEASES    OF    THE    MIND    AND  NERVOUS    SYSTEM,    NEW  YORK 
POST-GRADUATE    MEDICAL    SCHOOL   AND    HOSPITAL 


WILLIAM  A.  WHITE,  M.D. 

SUPERINTENDENT     OF     THE     GOVERNMENT     HOSPITAL     FOR     THE     INSANE,     WASHINGTON,     D.    C. 

PROFESSOR    OF    NERVOUS   AND  MENTAL  DISEASES,    GEORGETOWN  UNIVERSITY;  PROFESSOR 

OF    NERVOUS   AND    MENTAL    DISEASES,    GEORGE    WASHINGTON    UNIVERSITY,    AND 

LECTURER    ON    PSYCHIATRY,    U.    S.    ARMY   AND    U.    S.    NAVY 

MEDICAL    SCHOOLS 


ILLUSTRATED   WITH    331    ENGRAVINGS  AND   11    PLATES 


LEA   &   FEBIGER 

PHILADELPHIA  AND  NEW  YORK 
1915 


■^  1 3^f  1 


Entered  according  to  the  Act  of  Congress,  in  the  year  1915,  by 

LEA  &   FEBIGER, 
in  the  Office  of  the  Librarian  of  Congress.    All  rights  reserved. 


PREFACE. 


The  diseases  of  the  nervous  system  are  no  longer  compassed  by  a 
description  of  the  gross  lesions  of  the  brain,  spinal  cord,  cranial  and 
peripheral  nerves.  The  more  limited  symptomatology  of  disorders  of 
these  structures,  which  in  this  work  has  been  called  sensori-motor 
neurology,  has  been  expanded  in  two  directions — in  one  by  the 
increase  in  our  knowledge  of  the  historically  oldest  portion  of  the 
nervous  system,  namely,  the  sympathetic  and  autonomic  (vegetative) 
nervous  system  and  in  the  other  by  the  increase  in  our  knowledge 
of  the  mechanisms  that  operate  at  the  psychic  or  mental  levels. 

The  vegetative  nervous  system  is  in  close  functional  relations  with 
the  endocrinous  glands,  and,  although  some  of  the  endocrinopathies 
may  ultimately  turn  out  not  to  be  exclusively  nervous  affections,  still 
these  organs  of  internal  secretion  are  so  closely  related  from  all  points 
of  view,  embryological,  anatomical,  physiological,  pathological,  and 
pharmacodynamic,  with  the  vegetative  nervous  system  that  their  dis- 
ordered functions  must  needs  be  considered  in  a  work  dealing  with 
the  diseases  of  the  nervous  system.  The  symptomatology  of  this 
region  constitutes  the  borderland  of  neurology  and  internal  medicine. 

At  the  highest  level  stand  the  mental  mechanisms  in  which  action 
receives  a  symbolic  representation.  Here  the  nervous  system  is  also 
the  medium  through  which  that  form  of  physiological  or  pathological 
activity  called  conduct  is  brought  about.  These  mechanisms,  while 
operating  consciously,  largely  through  the  sensori-motor  channels  of 
adjustment,  are  also  intimately  related  to  the  vegetative  levels  where 
through  the  emotions  they  act  unconsciously. 

The  authors  have  kept  in  mind  the  concept  of  the  individual  as  a 
biological  unit  tending  by  development  and  conduct  tov/ard  certain 
broadly  defined  goals  and  have  considered  the  nervous  system  as 
only  a  part  of  that  larger  whole.  The  part,  however,  partakes  of  the 
unity  of  the  whole  and,  so  far  as  possible,  the  attempt  has  been  made 
to  arrange  the  diseases  of  the  nervous  system  in  accord  with  this 
evolutionary  concept. 


iv  PREFACE 

For  practical  purposes  and  for  the  reasons  stated  the  work  has 
therefore  been  divided  into  three  parts  deaHng  respectively  with  the 
vegetative,  the  sensori-motor,  and  the  psychic  levels,  the  reactions  in 
all  of  which  come  to  pass  through  the  medium  of  the  nervous  system. 

Man  is  not  only  a  metabolic  apparatus,  accurately  adjusted  to  a 
marvelous  efficiency  through  the  intricacies  of  the  vegetative  neuro- 
logical mechanisms,  nor  do  his  sensori-motor  functions  make  him 
solely  a  feeling,  moving  animal,  seeking  pleasure  and  avoiding  pain, 
conquering  time  and  space  by  the  enhancement  of  his  sensory  possi- 
bilities and  the  magnification  of  his  motor  powers;  nor  yet  is  he  exclu- 
sively a  psychical  machine,  w^hich  by  means  of  a  masterly  symbolic 
handling  of  the  vast  horde  of  realities  about  him  has  given  him  almost 
unlimited  powers.  He  is  all  three,  and  a  neurology  of  today  that  fails 
to  interpret  nervous  disturbances  in  terms  of  all  three  of  these  levels, 
takes  too  narrow  a  view  of  the  function  of  that  master  spirit  in  evolu- 
tion, the  nervous  system. 

For  these  reasons  the  treatise  has  been  called  primarily  a  work  on 
the  diseases  of  the  nervous  system  rather  than  two  books,  one  on 
neurology  and  one  on  psychiatry,  which  would  perpetuate  a  distinction 
which  the  authors  believe  to  be  wholly  artificial. 

S.   E.   J. 

W.  A.  W. 

New  York  and  Washington,  1915. 


CONTENTS. 


CHAPTER   I. 
Methods  of  Neurological  Examination    ....       17 

CHAPTER  II. 
Mental  Examination  Methods 66 


PART  I. 
PHYSICO-CHEMICAL   SYSTEMS. 

(Vegetative  or  Visceral  Neurology.) 


CHAPTER  III. 

The  Autonomic  and  Sympathetic  Systems — The  Internal  Secretions. 

Anatomy  of  Sympathetic  and  Autonomic  Divisions 82 

Special  Pathology 85 

Eye  Sympathetic 85 

Tear  Glands 87 

Mucous  and  Salivary  Glands 87 

Neck  Sympathetic 88 

Esophagus 89 

Stomach  and  Intestines 90 

Rectum 92 

Genito-urinary  System 94 

Respiratory  Apparatus 97 

Vascular  Apparatus 98 

Bloodvessels:    Vasomotor  Neuroses 100 

1.  Tonic  Hyperemias 101 

Erythromelalgia 101 

2.  Spastic  Anemic  Group 102 

Raynaud's  Disease 102 

Intermittent  Claudication 103 

Ophthalmic  Migraine 104 

Periodic  Palsies 121 

Ophthalmoplegic  Migraine 121 

3.  Vasomotor  Irritability  Group 123 

Angioneurotic  Edema 123 

Vegetative  Skia  Disorders        128 

Multiple  Gangrene  of  the  Skin 130 

Sweat  Secretory  Mechanism 130 


vi  CONTENTS 

CHAPTER  IV. 
The  Endocrinopathies. 

Internal  Secretions.     General  Considerations 132 

The  Thyreopathies;   Thyreoses 133 

Hypothyreoses 133 

Myxedema .  133 

Cretinism 137 

Sporadic  Cretinism 137 

Cretinoid  Degeneration 139 

Goitre 140 

Goitre  Heart .140 

Endemic  Cretinism 140 

Endemic  Deaf-mutism 142 

Hyperthyreoses 144 

Exophthalmic  Goitre 144 

Parathyroid  Syndromes 152 

Tetany 152 

Diseases  of  the  Hypophysis — Pituitary 161 

Hyperpituitarism 161 

Acromegaly  and  Gigantism 161 

Hypopituitarism ■.      .      .      .  162 

Dy spit uitar ism 163 

Polyglandular  Syndromes 165 

Pineal  Syndrome 165 

Adiposis  Dolorosa 166 

Genital  Syndromes  .      .      .      .     ' 167 

Agenitalism,  Hypergenitalism,  Hypogenitalism 167 

Status  Thymolymphaticus 167 

Addison's  Disease 167 

Pancreatic  Syndromes 172 

Liver  Syndromes 173 

Progressive  Lenticular  Degeneration 173 

Muscle  Syndromes 174 

Myasthenia  Gravis 174 

Thomsen's  Disease — Myotonia  Congenita 176 


PART   II. 

SENSORI-MOTOR  SYSTEMS. 

(Sensori-motor  Neurology.) 


CHAPTER  V. 

Cranial  Nerves. 

I.  Olfactory  Tract  Disorders 179 

II.  Diseases  of  the  Optic  Pathways 183 

Retinitis 183 

Optic  Nerve •      ••  ^^^ 

A.  Axial  Neuritis 186 

B.  Interstitial  Peripheral  Neuritis 189 

C.  Diffuse  Neuritis •     . 190 

Thalamic  and  Cortical 194 


CONTENTS  vii 

III,  IV,  VI.  Diseases  of  the  Oculomotor  Nerves 197 

Third  Nerve  Palsies 197 

Clironic  Progi-essive  Eye  Palsies 202 

Fourth  Nerve  Palsies 202 

Sixth  Nerve  Palsies 202 

Combined  Palsies 203 

V.  Diseases  of  the  Trigeminal  Nerve 209 

Motor  Part    . 209 

Sensory  Part 211 

Progressive  Facial  Hemiatrophy 215 

VII.  Diseases  of  the  Facial  Nerve 215 

Cortical  Facial  Palsies 218 

Pontine  Facial  Lesions 219 

Peripheral  Facial  Palsies 219 

VIII.  Diseases  of  the  Auditory  and  Vestibular  Pathways 222 

Auditory  Nerve 222 

Vestibular  Nerve 225 

Vestibular  Vertigoes 228 

XI,  XII.  Diseases  of  the  Spinal  Accessory  and  Hypoglossal  Nerve     .      .  233 

Accessorius 233 

Hypoglossus 234 

Speech  Disturbances ^ 237 

Aphasias 241 

Writing  Disturbances 244 

CHAPTER  VI. 

Affections  of  the  Peripheral  Nerves:  Sensory  and  Motor 

Neuralgias 245 

Special  Localized  Forms  of  Neuralgias 255 

Trigeminal  Neuralgia,  Tic  Douloureux  .........  255 

Cervicd-occipital  Neuralgia 259 

Diaphragmatic  Neuralgia , 260 

.  Brachial  Neuralgia 260 

Intercostal  Neuralgia 263 

Lumbar  Plexus  Neuralgia 264 

The  Sciaticas 264 

Lumbo-abdominal  Neuralgia 270 

Testicular  Neuralgia 270 

Crural  Neuralgia 270 

Femoral  Neuralgia 271 

Obturator  Neuralgia 271 

Neuralgias  of  the  Pudendal  Plexus 271 

Neuralgias  of  the  Coccygeal  Plexus 272 

Herpes   Zoster;     Shingles;     Zona.      Radiculo-ganglionic   Syndrome    (Acute 

Posterior  Poliomyelitis) 272 

Radiculitis 274 

Neuritis 278 

Polyneuritis,  Multiple  Neuritis ■ 279 

Alcoholic  Multiple  Neuritis 281 

Lead  Neuritis 283 


viii  CONTENTS 

Neuritis : 

Arsenical  Neuritis 284 

Infectious  Disease  Types 285 

Plexus  Palsies 286 

Brachial  Plexus  Palsies 286 

Total  Brachial  Palsy -  .  ■ 287 

Inferior  Root  Type 288 

Superior  Brachial  Plexus  Palsy 288 

Mixed  Types 289 

Lumbosacral  Plexus 291 

Peripheral  Palsies 292 

Peripheral  Palsies  due  to  Nerve  Injuries 293 

Spinal  Accessory  Nerve 300 

Cervical  Pub 300 

The  Long  Thoracic  Nerve        301 

The  Circumflex  Nerve 301 

Ulnar  Nerve 301 

Musculospiral  Nerve .■ 302 

External  Popliteal  Nerve 302 

External  Cutaneous  Nerve 302 

CHAPTER  VII. 

Lesions  of  the  Spinal  Cord. 

Acute  Polioencephalomyelitis 305 

Primary  Progressive  Muscular  Atrophies 319 

Group  1.  The  Progressive  Nuclear  Atrophies:     (1)  Spinal,  (2)  Bulbar, 

and  (3)  Mesencephalic  Forms 320 

Chronic  Poliomyelitis 320 

Aran-Duchenne  Type.     Progressive  Muscular  Atrophy  321 

Infantile  Hereditary  Forms 323 

Bulbopontine  Types.    Chronic  Progressive  Bulbar  Palsies  .  323 
Pontomesencephalic  Forms — Chronic  Progressive   Ophthalmo- 
plegia        324 

Group  2.    The  Neural,  Neuritic,  or  Spinal  Neuritic  Atrophies      .      .      .  325 

Peroneal-forearm  Type 325 

Tabetic  Type 327 

Group  3.  The  Muscular  Dystrophies  or  Myopathies 328 

Amyotrophic  Lateral  Sclerosis 337 

Fracture  and  Dislocation  Syndromes 341 

Compression  of  the  Cord 348 

Bone  Hypertrophies 348 

Tuberculosis  (Caries) 348 

Spinal  Cord  Tumors 349 

Lateral  Sclerosis  Group 351 

Combined  Scleroses.    Combined  Degeneration 353 

Combined  Sclerosis 354 

Combined  Sclerosis  in  Paresis 355 

Toxic  Forms 355 

Senile  Forms 355 

Syringomyelia 355 

Multiple  Sclerosis 368 


CONTENTS  IX 

CHAPTER  VIII. 

Diseases  of  the  Pons,  Brain  Stem  and  Midbrain. 

Medullary  Syndromes 385 

Pontine  Syndromes 387 

Crural  Syndromes 389 

Mixed  Syndromes 391 

CHAPTER  IX. 

Cerebellar  Syndromes. 

Chief  Syndromes 410 

Inferior  Cerebellar  Peduncle — Corpus  Restiforme 410 

Lesions  of  the  Middle  Cerebellar  Peduncle 411 

Lesions  of  the  Superior  Cerebellar  Peduncles 411 

Cerebellar  Aplasia 411 

Hemorrhage  of  Cerebellum 415 

Cysts  of  Cerebellum 416 

Tumors  of  Cerebellum 416 

Abscess  of  Cerebellum 419 

Posterior  Fossa  Syndromes 420 

Pontocerebellar  Angle  Syndromes 421 

CHAPTER  X. 

Paralysis  Agitans,  Chorea,  and  Related  Disorders. 

Paralysis  Agitans 423 

Dystonia  Musculorum  Deformans 431 

The  Choreas 433 

Chorea  Minor !      ....  434 

Huntington's  Chorea 437 

CHAPTER  XI. 

Diseases  of  the  Meninges. 

Dural  Disease 441 

Meningeal  Apoplexy 441 

Inflammation  of  Dura 444 

Pachymeningitis  Externa 444 

Pachymeningitis  Interna  Simplex 444 

Pachymeningitis  Interna  Hemorrhagica 444 

Diseases  of  the  Arachnoid  and  Pia,  Leptomeningitis 446 

Acute  Leptomeningitides 446 

Infectious  Meningitis 450 

Epidemic  Cerebrospinal  Meningitis 450 

Tuberculous  Meningitis 452 

Chronic  Leptomeningitis 454 

Hydrocephalus 455 

CHAPTER  XII. 

Diseases  of  the  Brain. 

Encephalitis — Abscess  of  the  Brain 458 

Acute  Encephalitis 459 

Abscess  of  the  Brain 462 


X  CONTENTS 

CHAPTER  XIII. 

Diseases  of  the  Brain  {Continued). 

Vascular  Disturbances — Cerebral  Apoplexy 466 

Vascular  Instability .  468 

Anemia 468 

Hyperemia 468 

Cerebral  Arteriosclerosis 468 

Cerebral  Apoplexy 470 

Hemorrhage,  Thrombosis,  Embolism 470 

The  Thalamic  Syndrome 474 

Sensory  Changes  in  Cortical  Lesions,  Suprathalamic  Pathways  .  478 

Apraxia 484 


CHAPTER  XIV. 

Tumors  op  the  Brain. 

True  Tumors 493 

Infectious  Tumors 495 

Parasitic  Cystic  Tumors       .      .      .   ' 495 

Aneurismal  Tumors 495 

Frontal  Lobe  Tumors 501 

Central  Convolution  Tumors 503 

Parietal  Lobes  Tumors 504 

Temporal  Lobe  Tumors .      . 505 

Occipital  Lobe  Tumors 506 

Corpus  Callosum  Tumors 507 

Optic  Thalamus  Tumors 508 


CHAPTER  XV. 

Syphilis  of  the  Nervous  System. 

Syphilis  of  the  Cranial  Bones  Causing  Neurological  Symptoms      ....  523 

Syphilitic  Meningitis  of  the  Base 524 

Syphilitic  Meningitis  of  the  Convexity 526 

Cerebral  Syphilis 529 

Vascular  Types 529 

Parenchymatous  Types  (Paresis) '532 

Dementing  Forms 543 

Depressed  Forms 543 

Expansive  Forms 544 

Agitated  Forms 547 

Irregular  Forms      .      . 548 

Juvenile  Paresis •      •      ■      •  549 

Syphilitic  Psychoses 550 

Tabes         ■      •      • ^^^ 

Syphilitic  Meningomyelitis 573 

Congenital  or  Hereditary  Syphilis ■  577 


CONTENTS  XI 


PART   III. 


PSYCHIC  OR  SYMBOLIC  SYSTEMS. 

(Neuroses  and  Psychoses.) 


CHAPTER  XVI. 

The  Psychoneuroses  and  Actual  Neuroses. 

The  Psychoneuroses 598 

Hysteria 598 

Compulsion  Neurosis 605 

Anxiety  Hysteria 612 

The  Actual  Neuroses 613 

Anxiety  Neurosis 613 

Neurasthenia 620 

Mixed  Neuroses 623 

CHAPTER  XVII. 

Manic-depressive  Psychosis. 

Manic  Phase 634 

Depressive  Phase 640 

The  Periodical  Types 642 

The  Cyclothymias 645 

The  Mixed  States 647 

CHAPTER  XVIII. 

The  Paranoia  Group. 

Paranoia  of  Kraepelin 655 

Mixed  and  Aberrant  Forms 658 

Paraphrenias .......  659 

CHAPTER  XIX. 

Epilepsy  and  Convulsive  Types  of  Reaction. 

Classical  Epilepsy 667 

Attenuated  Forms.     Affect  Epilepsies 670 

Epilepsies  of  Gross  Brain  Disease 671 

CHAPTER  XX. 

Dementia  Precox  (Schizophrenia)  Group. 

Dementia  Simplex 682 

Hebephrenia 692 

Catatonia 694 

Paranoid  States 697 

Mixed  and  Atypical  States 698 


xii  CONTENTS 

CHAPTER  XXI. 

Infection — Exhaxjstion  Psychoses. 

Prefebrjle,  Febrile,  and  Postfebrile  Psychoses 712 

Exhaustion  Psychoses 714 

Collapse  Delirium 715 

Acute  Hallucinatory  Confusion  (Amentia) 716 

Typhoid  Psychoses 717 

CHAPTER  XXII. 

The  Toxic  Psychoses. 

Alcoholism 719 

Drunkenness 721 

Chronic  Alcoholism        ...,...>. 721 

Dehrium  Tremens 722 

Korsakow's  Psychosis 724 

Alcoholic  Hallucinosis 725 

Alcoholic  Pseudoparesis 726 

Alcoholic  Pseudoparanoia 726 

Alcoholic  Epilepsy 727 

Dream  States      . 727 

Dipsomania 727 

Opium 727 

Cocain 728 

Miscellaneous  Intoxicants 728 

Bromides 728 

Carbon  Monoxide 729 

Lead 729 

Mercury 729 

Uremia ' 730 

Diabetes  MelUtus 730 

Gastro-intestinal  Diseases 730 

CHAPTER  XXIII. 

Psychoses  Associated  with  Somatic  Disease. 

Apoplexy 732 

Traumatic  Psychosis 733 

Acute  Chorea        . 735 

Chronic  Chorea 736 

Paralysis  Agitans 737 

Multiple  Sclerosis 737 

Polyneuritis 738 

Pellagra 738 

Heart  Disease 738 

CHAPTER  XXIV. 

Presenile,  Senile,  and  Arteriosclerotic  Psychoses. 

The  Presenile  Psychoses 740 

Involution  Melancholia • 740 

Other  Psychoses  of  this  Period 744 


CONTENTS 


Xlll 


The  Senile  Psychoses 

Intermediate  Conditions 
Normal  Senile  Involution    . 
Simple  Senile  Deterioration 
Senile  Delirium  .... 
Presb5'oi)hrcnia  .... 

Alzheimer's  Disease   .... 

Arteriosclerotic  Psychoses     . 

Ai'teriosclerotic  Brain  Atrophy 
Subcortical  Encephalitis 
Perivascular  Gliosis 
Senile  Cortical  Devastation 


746 
746 
746 
748 
748 
748 
751 
752 
752 
752 
753 
753 


CHAPTER  XXV. 

Idiocy,  Imbecility,  Feeble-mindednesp,  and  Characterological  Defect 

Groups. 

Feeble-mindedness 760 

ImbeciUty 760 

Moral  Imbecility 760 

Idio-imbecility 760 

Idiocy 760 

Amaurotic  Family  Types 762 

Sclerotic  Types 763 

Cretinism 764 

Mongolism 766 

Hydrocephalic  Types 767 

Microcephalic  Types 767 

Paralytic  Types 771 

Traumatic  Types 771 

Epileptic  Types 771 

Inflammatory  Types 773 

Sensorial  Types 773 

SyphiUtic  Types       .      .  ■ 773 

Idiot-savants 774 

Mild  Grades  of  Defect .775 

Psychopathic  Constitution 777 

Anomalies  of  the  Sexual  Instinct .  778 

Quantitative  Anomalies 778 

Qualitative  Anomalies 778 

Masturbation 778 

Active  Algolagnia 778 

Passive  Algolagnia 778 

Homosexuality 778 

Narcissism 778 

Fetichism 778 

Bestiality 779 

Exhibitionism 779 

Necrophilia 779 

Index 781 


NERVOUS  AND  MENTAL  DISEASES. 


CHAPTER  I. 
METHODS  OF  NEUROLOGICAL  EXAMINATION. 

The  student  of  nervous  diseases  should  have  at  his  command  a 
practical,  systematic  series  of  methods  for  examining  his  patients. 
Such  a  scheme  of  case  examination  should  above  all  be  orderly,  and 
sufficiently  extensive  to  enable  him  to  make  a  rapid  sizing  up  of  the 
character  of  the  disturbance  under  investigation,  and  not  be  burdened 
with  details  for  which  an  application  will  be  found  in  only  the  rarest 
of  disorders. 

A  comprehensive  neurological  history  should  include  a  careful: 

1.  Family  history,  especially  fashioned  to  bring  out  factors  of 
heredity  and  environment  that  might  throw  light  upon  the  disorder 
under  question. 

2.  The  anamnesis,  or  patient's  own  {a)  early  history,  ih)  the  history 
of  the  development  of  the  disorder,  and  (c)  the  general  neurological 
and  general  clinical  examination. 

For  the  sake  of  convenience,  the  chief  factors  to  be  covered  are 
here  given  in  the  form  of  a  printed  questionnaire.  Such  printed 
schemes  have  their  advantages  and  their  disadvantages.  But  if 
carefully  and  thinkingly  followed  the  advantages  far  outweigh  the 
disadvantages.  For  the  beginner  in  neurology,  to  whom  this  book  is 
addressed  especially,  such  a  questionnaire,  well  grounded  in  the  mind, 
is  the  first  step  in  the  development  of  a  technique  of  case  examina- 
tion which  will  prove  invaluable.  It  is  not  thought  that  another 
form  may  not  prove  as  satisfactory,  but  some  definite  scheme  is 
indispensable. 

Questionnaire. — Naturally  the  heading,  size,  form  of  binding,  of 
ruling,  etc.,  will  be  determined  by  local  conditions  and  by  special 
needs.  The  definite  facts  which  are  noted  in  the  questionnaire  are 
not  by  any  means  all  of  the  facts  to  be  collected,  and  such  a  printed 
question  blank  is  given  purely  as  a  guide,  rather  than  as  a  finished 
product.  As  a  practical  blank  for  out-patient  dispensary  work  it  is 
invaluable,  and  also  for  note-taking  in  private  work.  In  hospital 
work  with  nervous  disorders,  where  it  is  hoped  that  autopsy  material 
may  put  opportunities  for  anatomical  research  in  one's  hands,  such  a 
2 


18 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


blank  is  naturally  too  didactic  and  inelastic.  Its  main  outlines, 
however,  are  still  desirable. 

The  initial  facts  are  the  name,  in  full,  maiden  name  in  married 
women,  age,  residence,  social  status,  married,  single,  widow,  divorced, 
and  race.  In  countries  where  immigration  is  active,  it  is  desirable  to 
know  not  only  the  birthplace  of  a  patient,  but  also  his  racial  stock. 

Family  History. — It  is  desirable,  in  studying  mental  disorders 
particularly,  to  get  a  family  history  as  well  as  an  objective  history, 
both  from  the  patient  and  from  other  members  of  the  family. 

The  first  factor  that  needs  to  be  investigated  is  that  of  heredity. 
Viewed  in  the  usual  loose  light  in  which  it  is  regarded,  the  facts  gathered 
concerning  heredity  are  usually  worthless;  they  are  worse,  they  are 
misleading  and  pernicious.  Better  no  facts  concerning  the  extremely 
complicated  question  of  heredity  than  false  ones.  In  ordinary  investi- 
gation the  research  need  not  be  exhaustive.  A  complete  consideration 
of  heredity  is  best  left  to  special  eugenic  students,  whose  business  it 


O 


5  Jm 


D  ®  [iH-®B-p®    ^ 


0      (N)  [n]   (N)  (n)  [n]  [i]      |i~5H 


N 


k 


Fig  .  1 . — Pedigree  chart  illustrating  how  two  defective  parents  naay  have  only  defective 
children.  A,  alcoholic;  C,  criminalistic;  D,  infant,  died  in  infancy;  F,  feeble-minded; 
A'^,  normal;   T,  tuberculous.     Goddard,  1910.     (Taken  from  Davenport.) 

has  become  to  properly  collate  and  weigh  the  mass  of  material  needed 
to  bring  out  salient  factors  in  the  heredity  problem.  The  main  facts 
noted  in  the  questionnaire  are  sufficient,  save  in  the  consideration  of 
certain  disorders  in  which  definite  heredity  factors  are  known,  such  as 
Huntington's  chorea,  Thomsen's  disease,  diabetes  insipidus,  deaf- 
mutism,  certain  optic  atrophies,  etc. 

Consanguinity  (Fig.  1)  in  the  parents  is  first  to  be  inquired  into; 
the  degree  of  relationship  should  be  explicitly  expressed. 

Consanguinity  may  or  may  not  have  any  relation  to  nervous  dis- 
orders; it  much  depends  on  the  stock.  Bad  factors  present  in  one 
or  another  may  be  absolutely  missed  in  the  descendants,  for  Mendelian 
laws  seem  fairly  well  established.  With  the  presence  of  dominant 
neuropathic  traits  in  both  ascendants,  the  chances  are  less  hopeful 
in  the  descendants — but  not  hopeless,  since  recessive  characters  are 
passed  on  as  well.  In  human  fecundity  but  few  of  the  ova  are  impreg- 
nated, and  each  ovum  is  undoubtedly  very  different.     The  chance 


No. 


Diagnosis :   Tabes. 


NAME,  J.  Jones.  Age,  4^        Occup.:  Clerk.  M.S.W.[D.\ 

Residence,  444  Spring  St.  Race,  U.S.  In  U.S.42yr. 

Date,  Jan.  5,  1910.  Examined  by  J. 


Heredity:  Parents  related :  0 

F.,d.  70;  apoplexy.     Mental:  0 

M.,  d.  64;  cancer.        Nervous:  0 

Children:  Only  child.  Epilepsy:  0 

TBC:  0  Diabetes:  0 

Alcohol:  0  Syph.:  0 

Birth:  Normal.  Eruptions:  f 

Walk:  N.    Speak:  N.       Read:  6  years. 
Children's  Diseases:  Measles. 
Enuresis:  0     Thumb:  0    Nail-biting,  etc. :  0 
Education:  P^ib.  Sch.,  High  Sch.,  to  17  years. 
Adult  Diseases: 

Syphilis:  26.  Treatment:  1  month. 

Shocks:  0  Internal:  Hg. 

Habits:  Ale:  +        Tob.:  +  + 

Sex:  Moderate. 

Trauma:  0 

Occupation  Toxemias:  0 

Convulsions  (injury,  tongue,  urine) :  0 
Constitution:  Healthy.  Weight:  150. 
Marriage:  At  32. 

Menses : 

Children:  1;  d.  in  infancy  {convulsions). 

Mis.:  2;  4  mos.  Dead:  0 


History: 

Chief  complaint:  Slight 
unsteadiness  in  gait  and 
severe  "rheumatic"  neu- 
ralgic pains  in  lower  ex- 
tremity for  past  four 
years.  Five  years  ago 
had  a  transitory  attack 
of  dizziness,  with  double 
vision  and  an  eye  palsy, 
cross  eye,  which  lasted 
two  months. 

Occasional  weakness  in 
bladder,  dribbling.  Par- 
esthesia occasional,  etc. 


First  page  of  Questionnaire:  Made  out  from  examination  of  a  patient  with  Tabes. 


STATUS  NERVOSUM 


Cranium:  Hor.,  N. 
Sensibility:  O.K. 
Asymmetry:  0 

fOl.  ter.  O.K 

lAsafet.  O.Zi, 
fR.  20/40 


Bin.,  N.     Vert.,  N. 
Deformity:  0 


I.  Smell: 


11. 


III. 


V. 


Sight: 

L.  20/100 

Hemianopsia :  0  Scotomata :  0 

Fundus:  0  Fields:  Limited;  con. 

IV.  VI.  Eye  movements:  O.K. 
Nystagmus :  0  Palp.  fiss. :  R^L. 

Diplopia:  0;  5  years  ago. 
Pupils:  Ry-L,  9  and  4  mm. 

[Light:  Lost  L;  dim.  R. 

Accom. :  O.K. 

j Symph. :  Dim.  R^L. 

[Consensual:  Lost  L-\-R. 


Reflexes : 


Motor:  O.K. 
Sensory:  O.K. 
Tender  spots  :6' 


Jaw  jerk:  O.K. 
Cornea:  O.K. 
Conjunctiva:  O.K. 

VII.  At  rest:  O.K.  R=L. 

Forehead:  0.  K.      Eyes  closed:  0.  K. 
Teeth:  O.K.  Whistle:  O.K. 

Involuntary:  O.K.  Rosenbach:  O.K. 

VIII.  Hearing:  Dim.  Equihbrium:  O.iv. 
Rinne:  +  Weber:  + 

Tinnitus:  0 
IX.  X.  XL  XIL  Fauces:  O.X. 

Speech  .O.K.  Reflexes  :+0.K. 

Tongue: Straight.  Swallow:  O.iv. 

Scars:  0  Tremor:  0 

Cardiac:  0.  K.  Respiratory:  0.  K. 
Taste:  O.K. 

Shoulders:  0.  K.  Neck:  0. K. 


History — Continued 


STATUS  NERVOSUM 


Upper  extremity: 

Atrophy:  0 
Hypertrophy:  0 
Spasm:  0 

Hypotonus:  +  +      Twitching:  0 
Muscular  power:  Dim.    R=L. 
Dynanom. : 

Nerve  trunks:  Not  tender. 
Triceps  Rx. :  Dim.      Radix  Rx. :  Dim. 
Tremor:  0    Rest:  0  Static:  0 

Ataxia:  +    F.N.T.:  Atax.    F.F.T.:  Atax. 
Stereognosis :  O.K. 
Light  touch:  O.K.  Adiadokok:  0 
Position:  O.K.       Diapason:  Dim. 
Pain:  O.K.  Thermal:  O.K. 

Vasomotor:  0         Trophic:  0 
Trunk : 

Power:  O.K.  Deformity:  0 

Spine:  O.K. 

Epigastric  Rx. :  +   Abdom.  Rx. :  + 
Cremaster  Rx. :  +  Anal.  Rx.:  + 
Bladder:  Sluggish.  Rectum:  Sluggish. 
Light  touch  -.O.K.  Localization :  O.K. 
Deep  Sens.:  7) m.    Diapason:  Dim. 
Pain:  O.K.  Thermal:  O.K. 

EquilihiUnsteady.  AsyneTgia,:  +.    Slight. 
Vasomotor:  0         Trophic: 
Dermographia :  0 


History — Continued 


STATUS  NERVOSUM 


Lower  extremity: 

Atrophy:  +;  legs  flabby. 
Hypertrophy:  0 

Spasm:  0  Kernig:  0 

Hypotonus:  +  +        Tremor:  0 
Muscular  power:  Diminished. 
Synergistic  tests:  R=L. 
Patellar:  0    Achilles:  0      Clomis:  0 
Babinski:  0  Chad:  0         Opp:  0 
Nerve:  Tender.         Lasegue:  Tender. 
L.  touch:  Dim.        Pain:  O.K. 
Deep:  Dim.  Therm:  O.K. 

Localization:    O.K.  Position:  Impaired. 

Diapason :  Impaired. 
K.  H.  T. :  Atax.       Romberg:  +  +  + 
Gait :  Markedly  ataxic. 

Closed  eyes :  Made  worse. 
Asynergia:  O.K. 
Vasomotor:  0       Trophic:  0 
Status  corporis :  Medium  nutrition. 
Heart:  O.K.         Murmurs:  0 
Blood:  O.K.         Wassermann:  +  + 
Lungs:  O.K.        Arteries:  O.K. 
Abdomen:  O.i^.   Blood-pressure:  i^O. 
Liver:  O.K.  Skin:  O.K. 

Urine:  O.K.         Joints:  O.K. 
Cerebrosp.  fluid: 

Wass.:   ++  Cells:  60.  Globulin:  +  + 


Treatment : 

Intraspinous  injections 
of  salvarsanized  serum 
(Swift-Ellis).  Course  of 
mercurial  inunctions. 


FAMILY  HISTORY  23 

element  seems  too  great  to  estimate.  One  feature  of  consanguinity 
not  to  be  overlooked  is  that  many  relatives  (first  cousins,  second 
cousins)  marry  each  other  because  of  a  neuropathic  tendency.  It^4is 
not  impossible  then  that  the  evil  results  of  consanguinity  sometimes 
seen  is  a  direct  transmission  of  the  neuropathic  traits  that  were  pri- 
marily responsible  for  the  marriage.  At  all  events  the  studies  of 
Woods/  Coxr  Lundborg,^'  Punnett,''  Bateson,"^  Karplus/  and  the 
numerous  papers  of  Karl  Pearson/  all  give  hope  of  new  and  more 
definite  outlooks  in  the  study  of  the  relationship  of  heredity  todisease 
in  general,  and  of  disorders  of  the  nervous  system  in  particular. 

Abraham"^  and  others  have  given  some  suggestive  discussions  con- 
cerning the  tendency  for  related  neurotics  to  marry. 

Mental  Disorder.— The  presence  of  mental  disorder  in  the  direct 
ancestry  is  of  moment.  The  evidence  drawn  from  aunts  and]uncles  is 
of  value,  while  that  from  cousins  is  open  to  the  influence  of£another 
stock.     It  is  useless  to  record  the  fact  "insane"  without  any  definite 


II 


3|    4       5  16 

TTT       allN  allN  all  N     I      U^l    f 

I      w^mr    [ — 1    I — 1     '^m      V  y    1 — 1  numerous 

decendants 

all  normal 

IV 

Fig.  2. — Pedigree  of  a  family  showing  Huntington's  chorea.  Affected  persons  (indi- 
cated by  black  symbols)  are  always  derived  from  affected  parents.  From  original  data 
furnished  by  Dr.  S.  E.  Jelliffe;    Smi  family.     (Taken  from  Davenport.) 

knowledge  of  the  particular  mental  disturbance.  It  is  important  to 
reiterate  that  the  old  Morel,  Zeller  view,  that  all  mental  disturbances 
are  one  disease  with  different  stages  is  absurd.  Insanity  as  a  disease 
entity  has  no  existence.  One  should  be  as  specific  as  possible,  for 
there  is  a  distinct  tendency  for  special  types  to  be  passed  down  as 
such,  a  fact  that  only  the  more  fundamental  conceptions  of  Kraepelin 
have  permitted  to  be  verified.  Mental  peculiarities  should  be  noted: 
great  avarice,  originality,  queerness,  eccentricities,  great  capability, 
marked  incapacities,  temperamental  peculiarities,  inability  of  husband 

'  Heredity  in  Royalty,  1906.  ^  Psychiatrischen  Neurologische  Bladen,  1907. 

3  Ueber  Degeneration,  1901.  ^  Mendelism  in  Relation  to  Disease. 

6  Brain,   1906. 

^  Zur  Kenntniss  der  Variabilitat  und  Vererbung  am  Zentralnervensystem,  1907. 
'  Biometrica. 

s  Die  Stellung  der  Verwandtenehe  in  der  Psychologie  der  Neurosen,  Jahrbuch  f. 
Psychoan.  u.  Psychopath.  Forschungen,  1909. 


24  METHODS  OF  NEUROLOGICAL  EXAMINATION 

and  wife  to  get  along,  suicides  (valuable  as  evidence  only  if  cause  be 
known),  all  these  may  be  of  considerable  service  in  offering  a  clue  to 
many  difficult  cases,  in  psychoneuroses  and  psychoses  particularly. 
The  occurrence  of  the  hysterical  reaction  in  the  parents,  or  in  the 
brothers  or  sisters  should  not  be  overlooked,  but  deductions  therefrom 
should  be  founded  on  precise  criteria,  not  haphazard  guessing. 

Definite  nervous  disorders  that  need  investigation  in  the  ancestry 
are,  so  far  as  known,  but  comparatively  few.  The  more  important 
are  tics,  myoclonias,  Huntington's  chorea,  Thomsen's  disease,  myop- 
athies, certain  congenital  brain  defects,  as  cerebellar  defects,  speech 
defects,  skin  defects,  optic  defects  (color  blindness).  Careful  history 
taking  will  undoubtedly  reveal  other  defect  neuroses,  for  the  very 
fundamental  study  of  Karplus  (loc.  cit.)  has  shown  how  brain  form, 
brain  cytotectonic,  cord  form,  cord  myelotectonic,  etc.,  are  directly 
transmitted.  Certain  occupation  neuroses,  some  migraines,  many 
eye  strains,  etc.,  are  possibly  due  to  the  continuous  transmission  of 
structural  variations.^ 

Eyileysy. — The  occurrence  of  epilepsy  as  epilepsy  means  little. 
Epilepsy,  like  many  another  disease  so-called,  is  resolvable  into  many 
different  factors.  The  convulsive  seizure  is  only  an  end  result  of  a 
vast  variety  of  antecedent  events  concerning  but  few  of  which  can 
one  posit  any  hereditary  factors. 

Alcoholism. — Alcoholism,  if  excessive,  should  never  be  overlooked. 
If  possible  the  cause  should  be  recorded,  for  in  the  last  analysis  alcohol- 
ism is  to  be  regarded  as  the  individual's  attempt  to  escape  certain 
difficulties.  Alcoholism  may  mean  hysterical  or  compulsive  reactions. 
Periodicity  in  drinking  should  be  carefully  inquired  into,  particularly 
in  its  relation  to  the  cyclothymic  constitution. 

Migraine. — The  hereditary  factor  in  migraine  is  much  exaggerated. 
The  extreme  frequency  of  the  disease  has  served  to  bring  about  this 
confusion.^ 

Tubercidosis  and  Diabetes. — Tuberculosis,  diabetes,  tendency  to 
arthritic  disturbances  are  factors  in  heredity,  the  exact  significance 
of  which  it  is  hard  to  estimate;  tuberculosis  especially,  because  of  its 
extreme  frequency.  The  presence  of  diabetes  in  a  parent  should  not 
be  overlooked.  It  seems  to  play  a  large  role  in  nervous  and  mental 
disorders. 

Syphilis. — Syphilis  as  an  antecedent  factor  should  never  be  disre- 
garded. Not  only  does  one  meet  with  congenital  tabes,  paresis,  hydro- 
cephalus, optic  atrophies,  deafness,  etc.,  but  evidence  is  accumulating 
that  reinforces  the  belief  that  a  syphilitic  heritage  is  responsible  for 
much  nervous  and  mental  disorder,  of  a  less  tragic  though  perhaps 
more  annoying  character  than  those  first  mentioned.  Objective  evi- 
dences of  a  syphilitic  inheritance  should  be  looked  for.     Teeth,  bony 

'  Compare  Adler,  Die  Mindewertigkeit  der  Organen. 

2  See  chapter  on  Migraine  in  Osier,  Modern  Medicine,  191.5,  second  edition,  vol.  6. 


FAMILY  HISTORY 


25 


formation,  pelvic,  chest,  and  cranial  contours,  etc.  Wassermann 
tests  of  the  blood  of  suspected  parents  may  even  be  necessary  to 
clear  up  a  diagnosis,  say  of  a  meningeal  disturbance  of  hidden  etiology 
in  an  infant,  child,  or  even  young  adult.  The  evidence  of  congenital 
Wassermann  reactions  is  still  too  undecided  at  this  date  to  permit 
one  to  be  satisfied  with  the  results,  especially  if  negative,  obtained  from 
the  blood  of  the  patient. 

The  objective  examination  passes  on  to  the  birth  of  the  patient. 
Was  it  normal,  or  instrumental,  or  of  excessive  length?  Hemorrhage, 
accidents,  or  pressure  palsies  may  thus  receive  their  interpretation. 

Did  the  child  learn  to  walk  at  a  normal  period,  i.  e.,  from  nine  to 
fifteen  months  (Preyer),  and  if  not  were  there  definite  facts— excessive 
weight,  intercurrent  disease— to  explain  the  tardiness?     Was  speech 


Fig.  3. — Congenital  syphilis  "unto  the  third  generation."  SyphiUtic  father  of  first 
generation.  Second,  three  premature  births,  two  dead  children,  and  one  dying  at 
sixteen  months.  Congenital  syphilitic  next,  with  Hutchinson  triad.  This  patient 
married,  and  had  a  child  with  snuffles,  who  died  at  age  of  six  weeks.  Remaining  sister 
had  infantile  hemiplegia,  Hutchinson  teeth,  and  keratitis.     (Mott.) 

acquired  early  or  late?  and  did  the  child  learn  to  read  at  a  normal 
period  (five  to  eight  years,  according  to  opportunities  afforded)? 
These  facts  are  of  much  importance,  especially  in  estimating  mental 
capacity,  and  for  young  children  probabilities  of  development  and 
the  need  for  special  training.  Children  who  learn  to  walk  and  talk 
as  late  as  twenty-six  to  thirty-eight  months  respectively  often  remain 
very  backward.  The  relations  of  speech  to  mental  development  are 
extremely  close.  Special  tests  as  the  Binet-Simon  scale  are  valuable 
in  placing  the  intellectual  age  of  the  child.  (See  Chapter  on  Mental 
Examination.) 

Difficulties  in  teething,  especially  when  attended  with  convulsions, 
should  be  noted.     The  rachitic  chest  form  should  not  be  overlooked. 

Concerning  children's  diseases,  much  can  be  said.     Severe  measles, 


26  METHODS  OF  NEUROLOGICAL  EXAMINATION 

or  scarlet  fever,  or  other  disorders  may  lay  the  foundation  of  a  later 
developing  epilepsy;  diphtheria  may  cause  various  forms  of  neuritis 
with,  at  times,  permanently  weakened  powers.  Tuberculosis  plays  a 
very  important  role.  The  importance  of  influenza  is  often  overlooked, 
while  the  relation  of  the  acute  streptococcic  infections  and  their 
resulting  joint  involvements  to  chorea  is  well  established.  Excessive 
fatigue,  or  exhaustion,  either  from  an  infectious  disease,  apart  from 
its  toxemia,  or  from  over-exercise  or  strain,  in  rapidly  growing  children 
bears  a  close  relation  also  to  choreic  reactions. 

Very  close  attention  should  be  paid  to  the  aural  affections  of  children, 
and  the  nasopharyngeal  cavities  scrutinized  for  adenoids,  or  other 
foreign  bodies  that  interfere  with  free  respiration,  sound  sleep  or  the 
proper  hygiene  of  the  nasopharyngeal  mucous  membranes. 

Intestinal  worms  should  not  be  overlooked.  They  may  be  the  cause 
of  infantile  convulsions  or  of  milder  neurotic  disturbances. 

The  occurrence  of  vertigo  should  be  inquired  into. 

Enuresis  should  never  be  overlooked.  If  continuing  past  the  third 
or  fourth  year  it  affords  valuable  evidence  of  a  neurotic  predisposition. 
Thumb-sucking,  nail-biting,  and  other  little  habits  may  be  included  in 
this  place.     Stammering  should  be  carefully  inquired  into. 

The  education  of  the  patient,  especially  if  a  mental  disorder  or  a 
psychoneurosis  is  under  investigation,  should  be  very  thoroughly 
gone  into.  A  knowledge  of  the  earliest  impressions  gained,  the 
picture  books  used,  the  principal  childish  associations  formed,  special 
tastes,  animosities  or  dislikes  are  essential  to  the  understanding  of 
the  neurasthenic,  hysterical  or  allied  reactions.  The  ideals  incul- 
cated, the  religious  and  ethical  training  gained  in  the  early  years 
usually  give  a  definite  stamp  to  the  personality  and  must  be  known  if 
the  adult  personality  is  to  be  understood.  The  grosser  factors  of  the 
classes  passed,  and  the  schooling  received  are  absolutely  essential 
in  estimating  the  grade  of  later  mental  capacity,  and  the  application 
of  intelligence  tests  in  the  study  of  the  psychoses  or  psychoneuroses. 

Adult  Diseases. — Syphilis  stands  in  the  first  rank.  Care  should  be 
exercised  in  obtaining  a  syphilitic  history.  Did  you  ever  have  a 
chancre?  is  the  usual  method  of  asking  the  question.  The  query, 
How  old  were  you  when  you  had  a  sore  on  the  penis  ?  although  perhaps 
more  abrupt,  will  give  a  higher  percentage  of  positive  answers,  espe- 
cially in  those  cases  where  its  previous  existence  is  largely  inferred,  as 
in  general  paresis  or  tabes.  If  the  direct  question  is  to  be  avoided, 
as  in  the  case  of  many  women,  married  or  otherwise,  the  questions 
concerning  syphilitic  symptoms  are  desirable.  The  presence  of  symp- 
toms of  continuous  sore  throat,  hair-falling,  etc.  A  physical  examina- 
tion for  mucous  plaques,  leukoplakia  of  the  mouth,  and  scars  on  the 
penis  should  never  be  omitted.  In  women  the  mucous  membranes 
of  the  cheeks  and  sides  of  the  tongue  should  always  be  examined. 

Further,  the  Wassermann  blood  reaction  should  be  obtained  in  all 
cases  where  any  doubt  exists.     Although  the  percentage  of  unknown 


ADULT  DISEASES  27 

syphilitic  infections  is  low,  nevertheless  such  exist.  A  single  Wasser- 
mann  test,  positive  or  negative,  is  not  conclusive,  and  great  care  should 
be  taken  in  the  choosing  of  a  proper  serologist.  The  subject  of  technic 
is  a  complicated  and  immensely  important  one.^ 

The  subsequent  history  of  the  syphilitic  infection  is  desirable  and 
the  character  and  length  of  time  of  treatment  should  be  recorded. 

Gonorrhea  is  not  unimportant.  It  is  of  special  relevancy  in  all 
arthritic  disorders,  in  choreas  (vaginal  discharge)  in  young  children, 
and  in  meningeal  excitements.     Gonorrheal  neuritis  is  known. 

Arthritis  in  its  various  forms  calls  for  careful  observation.  Here 
one  would  best  record  observations,  and  not  attempt  a  diagnosis  of 
the  joint  conditions. 

The  role  of  the  infections  in  mental  pathology  is  very  marked. 
Typhoid  fever  and  influenza  both  constitute  severe  infections  with 
marked  influence  on  nervous  tissues. 

Under  Shocks  is  included  sudden  mental  and  moral  influences, 
tending  to  disturb  the  emotional  life.  Loss  of  money,  of  parents, 
husband,  children,  or  loved  ones,  interference  with  one's  hopes  of  a 
career,  unfortunate  entanglements,  all  call  for  investigation.  The 
great  importance  of  emotional  disturbance  in  all  nervous  reactions 
should  be  borne  constantly  in  mind. 

As  to  habits,  particular  attention  should  be  directed  to  alcoholism. 
It  plays  a  most  important  role  in  diseases  of  the  nervous  system. 
There  is  much  divergence  of  opinion  as  to  what  may  constitute  alcohol- 
ism. Accurate  recording  of  the  exact  amounts  consumed  will  afford 
the  student  the  best  criteria  by  which  he  later  can  judge  for  himself 
from  his  own  carefully  kept  records.  Other  narcotics,  as  opium  and 
its  allies,  cocain,  the  alcohol  hypnotics,  bromides,  etc.,  call  for  record. 

Occupation  toxemias  should  not  be  overlooked.  They  are  daily 
assuming  increased  importance  in  America  by  reason  of  the  increasing 
number  of  dangerous  occupations.  Workers  in  lead,  arsenic,  mercury, 
copper,  pewter,  pottery,  dyes,  sulphur  compounds,  and  others  suffer 
often  from  obscure  symptoms,  due  to  chronic  poisonings.  Occupation 
fatigues  explain  many  neurasthenias. 

The  sexual  habits  should  be  inquired  into.  Liberal  indulgence  in 
masturbation,  etc.,  while  usually  self-corrective,  at  times  works 
havoc  with  the  nervous  system.  Sexual  repression  in  the  married  as 
well  as  the  unmarried  is  at  times  an  important  element  in  the  neuroses 
(anxiety  neurosis).  The  rarer  anomalous  sexual  impulses  call  for 
investigation  in  some  cases. 

Special  attention  should  be  paid  to  convulsive  seizures,  either 
occurring  in  the  young  or  in  adult  life.  Certain  facts  about  such 
convulsions  should  never  be  omitted.  These  are  the  presence  of 
dizziness,  or  of  objects  revolving  (direction  to  be  noted),  of  the  state 

1  Plaut.  Wassermann  Reaction  in  Neurology  and  Psychiatry,  Nervous  and  Mental 
Disease  Monograph  Series,  No.  5,  New  York,  1910;  Kaplan,  Serology  in  Nervous 
Diseases,  Philadelphia,  1914. 


28  METHODS  OF  NEUROLOGICAL  EXAMINATION 

of  consciousness;  whether  there  is  injury  to  the  body  during  such 
attacks;  if  the  tongue  be  injured;  if  urine  or  feces  be  voided  during 
the  attack,  and  if  there  be  amnesia,  complete  or  partial,  following  the 
attack. 

The  influence  of  violence  or  injury  to  the  body  is  often  of  extreme 
importance.  If  there  is  accompanying  mental  shock  the  fact  should 
not  be  omitted. 

The  general  constitution  of  the  patient — his  or  her  general  capacity 
for  work  and  fatigue — is  to  be  noted.  The  question  of  general  tem- 
perament, of  outlook  on  life,  may  be  tentatively  entered  in  this  place. 

In  the  case  of  women,  special  attention  should  be  directed  to  the 
menstrual  history.  The  number  and  character  of  the  births,  the 
health  of  the  children,  the  number  of  miscarriages  with  causes  should 
be  recorded. 

Subjective  History, — The  patient's  own  account  of  his  illness  can 
either  precede  or  follow  the  outlines  of  the  family  history  and  his 
general  previous  condition. 

It  is  advisable  for  the  patient  to  fix  as  nearly  as  possible  the  date 
of  the  onset  of  his  illness.  Certain  facts  which  may  or  may  not  have 
had  any  connection  with  the  malady  under  study  may  aid  in  fixing 
such  a  period  of  transition  between  health  and  sickness.  The  nature 
of  its  onset,  whether  acute  and  progressive,  or  acute  and  regressive, 
insidious  and  irregular,  or  very  severe  and  immediate.  What  did  the 
patient  notice  at  that  time?  Then  gradually  trace,  step  by  step, 
hour  by  hour,  day  by  day,  week  by  week,  or  year  by  year,  the  develop- 
ment of  the  disorder.  What  new  symptoms  have  been  added  to  the 
first — ^what  have  disappeared;  has  the  picture  remained  the  same,  or 
has  it  gradually  or  suddenly  altered? 

A  methodical  going  over  the  locomotor,  sensory,  emotional,  intel- 
lectual, skin,  digestive,  respiratory,  and  secretory  systems  should 
follow  the  patient's  own  account  of  the  difficulty.  Pains  felt  should 
be  roughly  charted  and  carefully  located.  If  certain  symptoms  have 
disappeared,  attention  should  be  paid  to  the  mode  of  their  onset  and 
of  their  departure.  It  is  important  to  ascertain  just  what  effect  the 
illness  has  upon  the  social,  familial,  physical  or  psychical  life  in  order 
to  estimate  the  severity  of  certain  symptoms.  Also,  has  the  patient 
consulted  other  phys'cians,  or  has  he  visited  institutions  or  other  than 
trained  medical  men  of  various  kinds?  What  was  the  treatment? 
Its  effect? 

A  thorough  subjective  anamnesis  is  one  of  the  most  difficult,  and  at 
the  same  time  most  important  features  of  a  neurological  or  mental 
examination,  particularly  the  latter.  If  the  gradual  evolution  of  the 
disorder  is  carefully  elucidated,  there  is  little  danger  of  going  wrong. 
If  one  hops  from  one  thing  to  another,  however,  mistakes  will  be 
frequent. 

The  Objective  Examination. — During  the  subjective  examination 
man}-  facts  concerning  the  general  attitude  of  the  patient  have  been 


CRANIAL  NERVES  29 

gained.  His  expression  and  carriage,  in  bed  or  able  to  be  about. 
The  character  of  his  intelHgence  and  the  responsibiUty  of  his  answers. 
Is  his  mind  clear  and  is  he  oriented? 

One  soon  determines  whether  one  has  a  purely  mental  case  to  deal 
with,  a  definite  neurological  picture,  or  a  borderland  or  combined 
condition.  The  systematic  neurological  examination  is  here  outlined 
first;  a  s}'stematic  mental  examination  is  given  in  a  following  section. 

As  has  already  been  noted,  a  scheme  is  of  great  advantage,  to 
beginners  particularly.  With  increasing  experience,  one  may  depart 
from  a  hard-and-fast  method  of  case-taking,  but  in  the  beginning 
the  student  should  habituate  himself  to  a  rigid  and  exhaustive  method 
if  he  would  avoid  careless  work. 

The  cranium  should  first  be  inspected.  Is  it  regularly  shaped  or 
is  there  assymmetry?  Measurements  of  the  vertical,  binauricular,  and 
horizontal  diameters  should  be  taken. 

The  position,  general  form,  and  character  of  the  ears  should  be 
noted,  and  the  arch  of  the  palate  observed.  The  occurrence  of  isolated, 
or  even  several,  so-called  signs  of  degeneration  may  be  recorded. 
There  is  no  necessary  connection  between  such  anomalies  and  nervous 
or  mental  disease.  They  are  found  in  superior  as  well  as  inferior 
deviates.  The  departure  from  the  average  is  worthy  of  record,  but 
the  hasty  generalizations  of  the  Lombroso  school  should  be  avoided. 
These  deviations  from  the  average  structures  will  be  discussed  later. 

Careful  and  thorough  percussion  of  the  skull  may  reveal  local 
points  of  tenderness  (brain  tumor),  etc.  The  presence  of  cicatrices 
(epilepsies),  depressions  (fractures),  or  abnormal  elevations  is  to  be 
.noted.  In  special  cases,  a-ray  examination  of  the  skull  is  of  great 
value,  and  should  always  be  made  for  suspected  fractures,  for  many 
brain  tumors  (acromegaly),  etc. 

The  development  of  the  two  sides  of  the  face  is  to  be  compared, 
the  width  of  the  nostrils  noted,  and  particular  attention  given  to  the 
character,  texture,  and  color  of  the  hair,  and  skin  of  the  face  and 
mucous  membranes. 

Cranial  Nerves. — These  should  be  systematically  tested.  I.  Smell. — 
There  are  no  satisfactory  quantitative  tests  for  smell  apart  from 
special  physiological  psychological  tests  that  are  of  service  in  research 
work  only.  The  smell  in  each  nostril  can  be  tested  separately,  prefer- 
ably by  some  well-known  substance  (oil  of  turpentine)  and  by  a 
substance  resembling  well-known  foods  (asafetida,  onions).  The 
nostril  of  one  side  is  stopped,  and  with  the  eyes  closed  the  patient  is 
asked  to  smell  from  a  bottle;  the  other  side  is  then  tested  with  the 
same  or  a  difl^erent  substance  and  comparisons  are  made.  Variations 
in  smell  are  very  common,  and  too  much  weight  is  not  to  be  given 
to  smell  tests.  Influenza  interferes  with  smell  tests.  Irritating 
substances,  like  ammonia,  etc.,  should  not  be  used.  A  search  for 
subjective  smell  disturbances  may  be  made  at  the  same  time.  Local 
conditions,  empyema  of  the  antrum,  etc.,  must  be  excluded.     Anosmia, 


30 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


unilateral  or  bilateral,  is  often  present  in  fractures  of  the  skull  and  in 
brain  tumors. 

II.  Eyes.—Sighi  should  be  tested  by  the  Snellen  or  other  type  cards. 
Myopias  and  astigmatisms  are  important  to  bear  in  mind  in  testing 


Jii;)Kt£y,_ 


Fig.  4. — Crossed  amblyopia  in  a  case  of  hysteria.     (Stewart.) 

the  sight  functions.  Hemianopsia  should  always  be  searched  for. 
It  may  be  done  rapidly  by  use  of  the  usual  finger  sight  test  and  if 
there  are  any  anomalies  careful  chartings  by  a  perimeter  should  be 
made.     Scotomata  are  sought  for  in  the  same  manner. 


liiffhlSya 


,80  •», 


oe  100 


Fig.  5. — Right  homonymous  hemianopsia  in  a  case  of  softening  of  the  left  occipital  lobe. 


Color  vision  is  important.  Colored  wools  in  the  full  daylight 
are  matched  as  to  shades.  In  color-blindness,  if  of  the  red-green 
variety,  gray-  or  straw-colored  wools  are  selected.  In  total  color- 
blindness the  individual  confuses  all  colors:  comparative  degrees  of 


CRANIAL  NERVES  31 

brightness  alone  are  distinguished.     A  number  of  ridiculous  blunders 
may  be  made  in  testing  for  color-blindness  with  the  wool-tests. 

The  field  of  vision  should  be  tested  with  avoidance  of  suggestive 
factors.  This  may  be  done  with  a  perimeter,  which  frequently 
introduces  the  error  of  suggestion,  or  the  rapid  finger  test  employed. 
The  patient  sits  about  three  feet  from  the  examiner.  Each  eye  is 
tested  separately.  The  patient  puts  his  hand  over  his  left  eye,  and 
looks  fixedly  at  the  examiner's  left  eye,  the  right  eye  being  closed. 
With  the  left  hand  held  midway  between  patient  and  examiner  some 
distance  to  one  side,  it  is  then  gradually  brought  toward  the  middle 
line,  the  fingers  being  waved  slightly.  The  patient  is  directed  to  say 
"now"  as  soon  as  he  catches  sight  of  the  slightly  waving  fingers. 
Four  axes  should  be  tested.  Variations  from  the  examiner's  own 
fields  can  be  noted.  A  square  of  white  paper  (1  cm.),  on  a  gray  card, 
may  be  used  instead  of  the  fingers.  For  a  general  test  of  the  color 
fields  squares  of  different  colors  may  be  used.  The  most  striking 
features  to  be  sought  for  are  hemianopsia,  temporal  or  nasal ;  concentric 
limitations,  irregular  limitations,  quadrant  hemianopsias,  psychic 
blindness. 

In  all  cases  the  fundus  should  be  examined.  A  knowledge  of  the 
eye-ground  changes  is  essential  for  good  neurological  investigation. 
Works  on  ophthalmology  must  be  consulted  for  the  many  anomalies, 
but  the  most  important  to  be  observed  are  signs  of  pressure,  of  atrophy, 
of  retinal  hemorrhage  or  congestion,  and  irregular  pallors  (temporal 
pallor  in  multiple  sclerosis),  edema,  choroiditis,  retinitis,  venous 
congestion,  arterial  occlusions,  etc.  Optic  atrophy  may  exist  without 
blindness. 

Third,  Fourth,  and  Sixth  Cranial  Nerves. — The  functions  of  the  third, 
fourth,  and  sixth  cranial  nerves  are  conveniently  tested,  first  together 
and  later  separately.  Ability  to  move  both  eyes  outwardly  (external 
rectus)  indicates  integrity  of  the  sixth  cranial  nerve.  If  the  eyes  can 
be  moved  freely,  amply  and  equally  upward,  downward,  and  inward, 
the  third  and  fourth  cranial  nerves  are  usually  intact. 

Having  seen  if  the  eyes  move  freely  in  all  direction,  nystagmus  is 
tested  for  under  the  same  conditions.  It  consists  of  a  slow  movement 
of  the  bulb  in  one  direction,  with  a  rapid  jerk  back  in  the  opposite 
direction.  Notes  on  the  direction  of  the  slow  and  rapid  movements 
should  be  made.  They  are  of  great  value  in  determining  labyrinthine 
and  cerebellar  nystagmus.  Nystagmus  may  be  present  on  central 
fixation,  or  only  become  apparent  as  the  eyes  are  directed  to  one  side. 
Slight  tremors  of  the  globes  on  extreme  lateral  position  may  be  of  small 
diagnostic  importance.  Nystagmus  should  be  tested  for  in  the  vertical 
and  horizontal  and  also  in  oblique  axes.  Rotatory  nystagmus  may 
be  looked  for. 

In  the  presence  of  a  nystagmus,  certain  supplementary  tests  are. 
advisable.  The  most  important  are  the  turning  stool,  and  hot  and 
cold  water  tests.     In  the  former  the  patient  is  seated  on  a  revolving 


32 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


office  chair,  and  turned  to  the  right  or  left  ten  revolutions,  with  moder- 
ate rapidity.  On  arresting  the  revolutions  careful  note  should  be 
made  of  the  direction  of  the  nystagmus  in  each  eye,  and  also  the  length 
of  time  it  persists.  The  head  may  be  held  erect,  and  bent  forward  or 
backward  to  test  each  semicircular  canal. 

Hot  and  cold  water  is  syringed  gently  into  the  external  auditory 
meatus  of  each  ear.  A  large  bulb  syringe  is  used.  Cold  causes  a 
definite  rotatory  nj^stagmus  toward  the  ear  not  irrigated.  The 
patient  also  has  vertigo  and  marked  disturbance  of  equilibrium.  If 
hot  water  be  used  the  quick  nystagmic  movement  will  be  toward  the 
ear  irrigated,  and  the  ataxia  is  different.  The  presence  of  labyrinthine 
trouble  or  cerebellar  disorder  involving  the  vestibular  apparatus  cause 
modifications  in  the  character  of  this  nystagmus  (see  Vestibular). 


Left  snpr. 
Rectus 


Right  siijjr. 
Rectus 


Left  infr. 
Rectus 


Riffht  infr. 
Rectus 


Left  infr. 
oblique 


Sight  infr. 
oblique 


Left  supr. 
oblique 


Right  supr, 
oblique 


A  B 

Fig.  6. — Werner's  "artificial  memory"  for  the  double  images  in  ocular  paralyses 
(Ophthalmic  Review,  1886).  A,  shows  the  position  of  the  images  in  paralysis  of  the 
recti  muscles;  B,  in  paralysis  of  the  oblique  muscles.  The  dotted  lines  indicate  "false" 
images,  the  thick  black  lines  "true"  images.     (Stewart.) 


Diplopia  is  next  tested  for.  The  patient  should  be  asked  if 
he  has  ever  seen  double  and  a  single  light,  or  one  finger  held  to  the 
right  or  left,  up  or  down,  used  to  test  if  double  vision  exists.  Should 
it  be  present  the  position  of  the  images  in  relation  to  one  another 
should  be  noted;  whether  they  separate  or  approach  as  the  candle  is 
farther  or  nearer,  and  a  red  glass  should  be  placed  before  one  or  the  other 
eye  to  determine  the  location  of  the  images,  and  their  relative  position. 

The  accompanying  schemes  are  of  value  in  memorizing  the  muscles 
involved  (Fig.  6) : 

Monocular  diplopia,  seeing  double  with  one  eye,  is  occasionally 
met  with.  It  is  due  to  gross  corneal  or  eye  defects,  occasionally  in 
central  scotomata,  but  usually  it  is  a  product  of  suggestion  in  hysteria. 

Pupils. — The  size  should  be  compared  and  noted.  r=l  :r>l,  l>r, 
and  a  rough  measure  given,  2,  3,  4  mm.,  as  the  case  may  be;  mydriasis, 


CRANIAL  NERVES 


33 


myosis.  The  form  and  the  presence  of  irregularities,  oval,  polygonal, 
aiid  implantation  noted.  Particular  attention  should  be  directed 
to  the  elimination  of  changes  due  to  drugs,  to  accidents  or  injuries, 
and  to  inflammatory  products. 

The  reactions  to  light  should  then  be  tested,  at  first  with  clear 
daylight,  the  patient  looking  at  some  distant  object.  In  bed  cases, 
electric  hand  lamps  are  very  serviceable;  with  these  the  light  is  best 
directed  somewhat  obliquely.     The  reaction  may  be  prompt  and  wide, 


Pyramidal 
Tracts 


Ganglion 
Siipremum 

■Sympathetic 


Fig.  7. — The  paths  of  the  chief  eye  reflexes:  (1)  Pupillary  reflex:  Retina;  optic;  X; 
corpora  quadrigemina ;  X;  oculomotor  nucleus,  oculomotor  nerve;  ciliary  ganglion; 
sphincter  iridis.  (2)  Closing  eyes  to  light:  Retina;  optic;  X;  corpora  quadrigemina, 
Meynert's  fibers;  X;  facial  nucleus;  facial  nerve;  lid  muscles.  (3)  Winking  on  ap- 
proach of  object:  Retina;  optic;  X;  corpora  quadrigemina  or  pulvinar,  external 
geniculate;  cortex;  pyramidal  tract;  X;  facial  nucleus;  facial  nerve;  lid.  (Same  paths 
also  in  2.)  (4)  Corneal  reflex:  Cornea;  trigeminus;  trigem.  nucleus;  facial  nucleus; 
facial  nerve;  lid.    (Lewandowsky :  Funktionen  d.  Centralnervensystem,  p.  122,  Fig.  29.) 


prompt  and  restricted,  slow,  sluggish,  diminished  or  absent.  Each 
eye  is  to  be  tested  separately,  and  then  the  consensual  test  applied 
by  illuminating  one  eye  and  noting  the  reaction  in  the  other. 

Wernicke's  hemiopic  phenomenon  should  be  sought  for  in  hemian- 
opsias. In  this  the  pupil  does  not  react  if  the  light  falls  upon  the 
blind  segment  of  the  retina. 

Tests  for  the  accommodation  reflexes  are  then  made.  The  patient 
looks  at  the  finger  as  it  is  moved  near  or  away  from  the  eye,  and  there 
is  corresponding  contraction  and  dilatation  of  the  pupils.  Here  the 
3 


34 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


reaction  may  be  prompt  or  slow,  with  slight  or  marked  amplitude. 
In  blind  people  the  thought  of  looking  at  their  own  nose  and  across 
the  room,  may  bring  out  the  reaction.  The  existence  of  a  lost  or 
partially  lost  light  reflex,  with  imimpaired  accommodation  reflex,  is 
known  as  the  Argyll-Robertson  pupil.  It  may  be  present  in  one  or 
both  eyes,  and  may  be  partial  or  complete.  It  is  due  to  a  dissociation  of 
the  sensorimotor  reactions,  and  may  be  present  in  a  variety  of  disorders, 
althougb  it  is  most  frequently  found  in  s\'philitic  disorders  (Fig.  7). 

The  mechanism  is  variously,  although  not  entirel}',  satisfactorily 
explained,  because  of  the  complexity  of  the  afferent  and  efferent 
fiber  tracts  and  their  connections  (Lewandowsky  scheme).     Marina's 

h^"pothesis  of  its  peripheral  origin 
(disease  of  ciliary  ganglion)  explains 
most  of  the  tabetic  and  paretic  cases, 
but  does  not  explain  some  of  the 
traumatic  or  mesencephalic  cases. 
Cajal's  scheme  is  as  follows: 

1.  Retinal    neuron   with  its  optic 
fibers  ramifying  in  the  anterior  cor- 
pora quadrigemina. 
^  !«»R«B«i^p  2.  The  mesencephalic  neuron  with 

^*X  .,  '       f  its   axis-cylinder    formation    of    the 

posterior  commissure. 

3.  The  neuron  of  the  interstitial 
nucleus  of  the  tegmentum  (calotte) 
with  its  collaterals  destined  to  the 
motor  nuclei. 

4.  The  neuron  of  the  bulbar  nuclei 
of  the  oculomotor  communis  et  ex- 
ternus  and  of  the  patheticus  with 
cylinder  axes  going  to  the  muscles  of 
the  eye. 

The  s}Tnpathetic  reflex  is  tested  by  pinching  the  skin  of  the  cheek 
or  some  other  part  of  the  body.  The  pupils  dilate  under  the  influence 
of  painful  stimuli. 

Ophthalmoplegia  externa  is  the  name  given  to  a  paralysis  of  the 
external  eye  muscles;  ophthalmoplegia  interna  to  those  of  the  pupil, 
which  is  widely  dilated  and  immobile  to  light  and  convergence.  Com- 
plete ophthalmoplegia  is  found  when  all  of  the  pupillary  phenomena 
are  absent  and  there  is  loss  of  all  eye  movements  with  ptosis. 

Ptosis  consists  in  a  drooping  of  the  upper  lid,  paralysis  of  levator 
palpebrae,  which  is  supplied  by  filaments  from  the  third  nerve.  Henne- 
berg's  reflex,  consisting  in  a  spastic  action  contraction  of  the  orbicularis 
oris  when  the  hard  palate  is  stroked,  may  be  mentioned  with  the  reflexes 
of  the  cranial  nerves. 

The  Fifth  (Trigeminns)  Nerve.- — The  motor  functions  of  the  fifth 
nerve  are  tested  by  having  the  patient  move  his  jaw  to  the  right  and 


Fig.  S. — Tabes   with  bilateral  ptosis 
Operated  upon  to  hold  eyelid  open. 


CRANIAL  NERVES  35 

left.  The  examiner's  hand,  exerting  contrary  pressure,  can  determine 
difFerences  in  innervation  (external  pterygoid,  temporals).  The 
finger  TVTapped  in  a  towel  can  be  used  to  bite  upon  to  determine 
variations  in  bite  (masseter,  temporal).  The  state  of  hardness  of 
the  temporals  and  masseter  muscles  can  be  directly  palpated.  The 
jaw  should  be  protruded  (internal  pterygoid). 

The  use  of  a  tuning  fork  of  low  pitch  is  useful  in  determining  loss 
of  the  function  of  the  tensor  tympani  muscle. 

In  one  sided  paralysis  of  the  motor  fibers  of  the  fifth  nerve,  the 
opened  jaw  deviates  to  the  paralyzed  side  by  the  action  of  the  sound 
external  pterygoid.  The  patient  chews  on  the  sound  side.  It  may 
or  may  not  be  accompanied  by  sensory  changes.  Elevation  of  the 
eyelid  on  strong  biting  is  a  frequent  associated  movement. 

The  jaw  reflex  may  be  tested  at  the  same  time.  With  the  mouth 
partly  open  a  pencil  or  flat  object  is  placed  upon  the  teeth  and  lightly 
tapped  with  the  hammer.  There  is  a  quick  contraction  of  the  mass- 
eters  and  temporals,  and  usually  an  associated  movement  of  closing  the 
eyelids. 

The  sensory  functions  of  the  fifth  nerve  demand  very  carefid  testing 
by  reason  of  its  wide  distribution. 

The  supra-orbital,  infra-orbital  and  mental  points  should  first  be 
pressed  upon  to  determine  the  degree  of  sensitiveness.  Then  the 
palpebral,  conjimctival,  and  corneal  reflexes  should  be  tested.  This 
is  best  done  with  a  long  pin  with  a  globular  glass  head.  With  the 
patient'  looking  away  from  the  examiner,  the  palpebral  margin  is 
touched  with  the  head  of  the  pLri,  then  the  conjimctiva,  and  finally 
the  choroid,  and  rimning  along  the  globe  over  the  cornea  the  effect 
is  noted.  Both  eyes  shoidd  be  compared  and  the  tear  secretion 
noted. 

The  amoimt  of  tear  secretion  may  be  measm-ed  by  hanging  two 
small  strips  of  litmus  paper  on  each  lower  lid,  by  bending  in  the  paper 
at  the  top  so  as  to  make  a  small  ledge  to  hang.  The  rate  of  moistening 
of  the  two  sides  will  show  quantitative  variations  in  the  amounts 
secreted. 

The  ordinary  sensibility  shoidd  be  tested  first  with  a  camel's-hair 
brush — ^the  two  sides  compared.  Then  with  the  point  and  head  of  a  very 
sharp  pin;  then  the  skin  shoidd  be  pinched  on  each  side  and  differences 
noted.  Deep  pressure  over  the  malar,  frontal,  and  jaw  bones  made  to 
determine  deep  pressure  sense,  and  the  use  of  hot  and  cold  test-tubes 
to  learn  if  variations  in  thermal  sense  exist.  Finally  a  slowly  \"ibrating 
diapason  shoidd  be  applied  to  the  jaw  bone  to  determine  its  bony 
sensibflity. 

The  Ulterior  of  the  mouth  and  the  surface  of  the  tongue  should  not 
be  neglected  in  these  tests,  and  special  care  shoidd  be  taken  in  outlining 
the  ear  areas,  and  changes  within  the  auditory  canal. 

Careful  inspection  of  the  teeth  should  not  be  omitted,  and  anomalies 
of  dentition  carefully  noted.     The  two  sides  of  the  bonv  structures 


36 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


of  the  face  should  be  compared  for  bony  atrophies  (hemiatrophy) 
or  hypertrophies  (acromegaly). 

The  Seventh  (Facial)  Nerve. — The  seventh  (facial)  nerve  is  a  mixed 
nerve.  The  state  of  the  musculature  at  rest  should  first  be  recorded. 
The  folds  and  the  angles  of  the  mouth  and  muscle-twitching  carefully 
noted.     The  patient  is  then  asked  to  wrinkle  his  forehead  (frown), 


Taste  &  Saliva 


-Sweat 
•  Tears 
-Taste  &  Saliva 


Post.  Auricular 
Nerve 


Fig.  9. — Diagram  of  facial  nerve,  showing  course  of  secretory  and  of  taste  fibers. 

(Stewart.) 

raise  the  eyebrows,  close  his  eyes  tight,  show  the  teeth,  whistle,  and 
puff  out  the  cheeks.  In  this  way  the  musculature  of  the  two  sides  can 
be  compared.  A  mild  joke  will  bring  out  the  emotional  contractions 
which  are  equally  important  to  note.  Some  individuals  can  move 
their  ears. 

Not  only  should  the  presence  of  paralysis  or  paresis  be  carefully 


CRANIAL  NERVES  37 

noted,  hut  spasms  observed  under  voluntary  and  emotional  reaction. 
Their  degree,  character,  and  intensity  should  be  carefully  recorded. 

Sensory,  secretory,  and  motor  functions  are  to  be  tested,  and 
many  difi'erences  are  to  be  noted  in  the  distribution  of  the  palsies 
according  to  the  location  of  the  lesion  of  this  nerve:  (1)  after  its  exit 
from  the  stylomastoid  foramen,  (2)  within  the  Fallopian  aqueduct, 
(3)  between  its  emergence  from  the  pons  and  the  geniculate,  or  (4) 
within  the  pons  (See  Fig.  9). 

The  Eighth  (Cochlear  and  Vestibular)  Nerves. — Here  two  entirely 
different  nerves  with  absolutely  different  functions  need  to  be  tested. 
They  are  the  cochlear  nerves  (hearing)  and  the  vestibular  nerves 
(equilibrium). 

Hearing  is  roughly  tested  most  satisfactorily  by  speech.  Having 
first  ascertained  that  the  auditory  canals  are  free  from  wax,  the  exam- 
iner, some  six  to  ten  feet  away,  pronounces  three  numbers,  asking 
the  patient,  who  has  one  ear  closed,  to  repeat  them  after  him:  the 
voice  is  then  raised  or  lowered,  and  the  distance  varied  to  determine 
the  hearing  capacity.  The  opposite  ear  is  tested  in  the  same  manner 
and  comparisons  made.  A  watch-tick  or  tuning-fork  may  also  be 
used.  Careful  tests  with  tuning-forks  and  whistles  are  needed  in 
complicated  cases. 

Bony  conduction  should  also  be  tested.  This  is  done  by  putting  the 
vibrating  tuning-fork  over  the  mastoid,  and  the  patient  indicates 
when  he  no  longer  hears  it,  the  fork  is  then  brought  to  the  auditory 
meatus  to  test  the  air  conduction.  Rinne's  test  is  positive,  i.  e.,  air 
conduction  present  after  loss  of  bone  conduction  is  the  normal  formula: 
the  negative  Rinne  indicates  middle-ear  disorder.  The  tuning-fork 
on  the  center  of  the  forehead  is  heard  in  both  ears  equally  under  normal 
conditions  (Weber).  In  middle-ear  afTections  it  may  be  heard 
unequally  on  the  two  sides.  Positive  Weber  {i.  e.,  louder  on  affected 
side)  with  negative  Rinne  is  largely  indicative  of  middle-ear  disorder. 
Deafness  due  to  central  disorder  is  usually  associated  with  other 
localizing  signs,  yet  it  may  be  an  isolated  phenomenon  of  beginning 
tumor,  encephalitic  process,  tabes,  multiple  sclerosis,  etc. 

Forks  of  very  slow  vibration  are  of  value  in  determining  the 
functional  capacity  of  the  stapedius  muscles.  Tests  with  continuous 
tone  series  following  Bezold's  methods  are  indicated  in  all  complicated 
cases,  since  defects  in  lower  or  in  higher  tone  perception  usually 
indicate  a  difference  in  the  site  of  the  lesion. 

Certain  patients  show  hyperacusis,  tinnitus.  Ringing  in  the  ears 
is  largely  a  subjective  sign.  It  is  an  evidence  usually  of  middle-ear 
or  of  cochlear  irritation.  The  sounds  vary  greatly.  They  may  be 
beating,  buzzing,  or  whistling,  and  may  at  times  be  the  point  of 
departure  of  illusions,  or  even  hallucinations.  The  pulsating  types  of 
tinnitus  are  usually  associated  with  the  heart  beat.  They  are  found 
in  certain  tumors,  in  aneurysms,  or  in  anxiety  states  with  cardiac 
irregularities.     Continuous  tinnitus,  low  pitched  or  high  pitched,  is 


38  METHODS  OF  NEUROLOGICAL  EXAMINATION 

the  more  common.  The  effects  upon  the  tinnitus  of  lying  down  and 
also  the  effects  of  certain  drugs,  amyl  nitrite,  etc.,  are  of  service  in 
differentiating  the  causes  and  probable  site  of  a  tinnitus. 

Vertigo. — This  belongs  largely  to  the  symptomatology  of  the  vestib- 
ular apparatus.  Equilibration  is  an  extremely  complex  adjustment 
involving  the  comparative  integrity  of  a  large  number  of  neurons  from 
the  periphery  to  the  coordinating  centers,  now  fairly  satisfactorily 
proved  to  reside  in  the  cerebellum.  The  vestibular  apparatus  is  the 
chief  ganglion  of  the  cranial  end  of  this  whole  apparatus  or  system 
termed  by  Sherrington  the  proprioceptive  system.  The  cerebellum 
is  its  chief  center. 

Tests  for  Equilibration. — The  more  standard  equilibration  tests  are 
the  Romberg,  and  the  Babinski  asynergic  tests.  The  Romberg  test 
consists  in  having  the  patient  stand  erect  with  closed  eyes,  with  heels 
and  toes  together.  Under  normal  conditions  there  should  be  only  a 
very  slight  swaying,  but  the  person  with  well-marked  Romberg  sways 
sideways,  or  backward,  or  forward,  or  may  even  fall  if  the  feet  are  not 
put  apart.  Slight  degrees  of  Romberg,  or  unilateral  localization  of 
Romberg,  may  be  brought  out  by  having  the  patient  stand  on  one  or 
the  other  foot.  Other  observations  of  the  Romberg  can  be  made  by 
having  the  patient  bend  forward  or  backward  or  sideways,  and  in  this 
way  quantitative  suggestions  may  be  received  as  to  the  localization  of 
the  fiber  tracts  involved. 

In  certain  cerebellar  disturbances  there  is  a  special  loss  of  equilibrium 
which  Babinski  has  termed  asynergia.  This  may  be  tested  in  a  number 
of  different  ways.  The  more  valuable  are  by  means  of  walking.  The 
patient  usually  has  a  great  deal  of  ataxia  and  walks  with  difficulty. 
If  supported  on  either  side,  it  is  noticed  that  in  walking  he  shows  a 
marked  tendency  to  walk  from  underneath  himself,  so  that  his  legs 
finally  are  put  out  far  in  advance  of  his  center  of  gravity.  The  same 
type  of  loss  of  cerebellar  equilibrium  may  be  demonstrated  by  having 
the  patient  stand  erect  and  then  slowly  bend  backward,  making  an 
arc  de  cercle.  In  the  normal  condition,  the  muscular  adaptation 
bends  the  knees  forward  and  the  individual  assumes  a  well-balanced 
position;  but  in  the  asynergic,  the  legs  are  held  straight,  the  trunk  is 
bent  backward,  and  the  patient  has  a  tendency  to  fall  backward. 

For  the  upper  extremities  a  similar  type  of  muscular  incoordination 
shows  itself  in  the  typical  diadokokinesis  tests.  These  tests  are  made 
by  having  the  patient  perform  certain  minute  complicated  movements 
very  rapidly,  such  as  quickly  pronating  and  supinating  the  hand  in  a 
semiflexed  position,  or  making  rapid  piano-playing  movements,  or 
sudden  flexions  and  extensions  of  the  arm  on  the  forearm.  In  involve- 
ments of  certain  of  the  cerebellar  tracts  usually  associated  with  equi- 
librium there  is  a  marked  inability  to  perform  certain  of  these  move- 
ments; they  are  done  clumsily,  irregularly  and  slowly,  depending 
on  the  grade  of  sensory  tract  involvement. 

Similar  tests  consist  in  having  the  patient  reproduce  on  a  piece  of 


CRANIAL  NERVES  39 

paper  single  lines  with  a  lead-pencil,  when  it  will  be  found  that  they 
almost  invariably  overrun. 

For  testing  the  trunk  the  patient  should  be  on  the  back,  with  the 
arms  folded,  and  with  the  legs  drawn  up  in  the  obstetrical  position. 
Here  considerable  variation  will  be  found.  The  normal  individual, 
if  on  a  fairly  hard  foundation,  can  equilibrate  fairly  well;  there  is  but 
very  little  swaying,  while  the  ataxic  patient  sways  considerably. 
Cerebellar  patients  also,  and  certain  types  of  frontal  cases,  show  what 
is  known  as  cataleptic  rigidity  in  this  position.  After  swaying  for 
a  certain  length  of  time  the  legs  and  body  steadily  become  fixed 
with  sufficient  rigidity  to  enable  them  to  be  photographed.  This  sign 
of  cataleptic  rigidity  is  considered  by  Babinski  as  characteristic  of 
cerebellar  defect. 

In  further  testing  equilibrium  the  gait  occupies  an  important  place. 
The  patient  should  be  directed  to  walk  along  a  crack,  or  a  straight 
line,  first  with  the  eyes  open;  he  should  be  asked  to  suddenly  reverse, 
inquiry  being  made  if  there  are  symptoms  of  vertigo  in  the  reversion 
either  to  the  right  or  the  left,  for  both  sides  should  be  tested,  and  then 
the  patient  should  be  directed  to  return  with  his  eyes  closed  when,  if 
an  abnormal  equilibrium  exists,  it  will  become  intensified,  and  stagger- 
ing to  the  right  or  to  the  left,  forward  or  backward,  will  be  noted  (latero- 
pulsion,  propulsion,  or  retropulsion) . 

The  subjective  sense  of  giddiness  is  often  extremely  complicated. 
Certain  patients  complain  of  objects  turning  about  them,  in  which 
case  the  direction  of  the  moving  object,  in  terms  of  the  hands  of  a 
clock,  should  always  be  noted.  Association  of  giddiness  or  vertigo 
with  eye  symptoms  is  very  widespread. 

The  Barany  tests  by  heat  and  cold,  by  compressed  air,  by  the 
revolving  chair,  have  already  been  mentioned  in  the  consideration  of 
nystagmus,  and  need  not  be  repeated  here.  They  are  primarily  tests 
for  the  labyrinthine  function,  the  nystagmus  being  only  an  accessory 
phenomenon. 

Examination  of  the  Pharynx. — Careful  note  should  be  made  of  the 
position  of  the  fauces  while  at  rest  and  during  phonation,  and  of  the 
movements  of  the  soft  palate  during  phonation.  The  pharyngeal 
reflexes  are  tested  by  touching  successively  the  right  and  left  pillar, 
and  the  vomiting  reflex  is  best  tested  by  irritating  the  back  of  the 
pharynx  with  a  wooden  spatula. 

While  under  normal  conditions  the  uvula  should  hang  in  the  middle 
line,  a  certain  amount  of  variation  in  position  is  very  frequently 
observed. 

While  the  pharynx  is  being  examined,  note  should  be  made  of  the 
pharyngeal  vault,  and  also  of  the  contour  of  the  posterior  pharyngeal 
walls.  Further,  the  integrity  of  the  superior  pharyngeal  muscles 
should  be  tested  by  having  the  patient  swallow,  and  noting  w^hether 
the  act  is  coordinated,  or  whether  there  is  regurgitation  through  the 
nose. 


40 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


Examination  of  Taste. — Taste  is  a  complex  function  and  utilizes  at 
least  three  different  nerves.  It  is  best  tested  by  solutions  which  are 
sweet  (sugar),  bitter  (quinine),  acid  (vinegar),  salty  (salt  solutions). 
The  solutions  should  be  kept  in  wide-mouthed  bottles,  and  are  applied 
in  small  quantities  by  means  of  a  glass  rod  applied  to  different  parts 
of  the  tongue  to  determine  the  functional  capacity  of  these  parts. 
Small  amounts  should  be  used,  and  it  is  best  to  reserve  the  bitter  test 
until  the  last,  commencing  with  the  sweet,  and  following  with  the  sour, 
the  salt,  and  the  bitter.  The  mouth  should  be  washed  out  between 
the  tests  with  the  different  substances  if  careful  records  are  to  be  made. 


Fig.  10. — Organic  laryngeal  paralyses.  (Barwell.)  I,  left  abductor  paralysis  during 
inspiration;  II,  left  abductor  paralysis  during  phonation;  III,  left  recurrent  laryngeal 
paralysis,  during  inspiration;    IV,  left  recurrent  laryngeal  paralysis,  during  phonation. 


Taste  can  also  be  tested  by  means  of  a  minimal  galvanic  current 
when  the  positive  pole  will  produce  an  acid  reaction,  the  negative 
pole  an  alkaline  or  salty  one. 

While  testing  for  taste  the  condition  of  the  secretion  of  the  buccal 
mucous  membrane  can  be  tested.  This  is  best  done  by  rubbing  the 
floor  of  the  mouth  with  a  glass  rod  and  noting  the  rapidity  of  excretion 
and  the  amount. 

Examination  of  the  Larynx. — This  naturally  has  to  be  carried  out 
by  means  of  a  laryngological  mirror,  when  mal-position  or  changed 
position  of  the  vocal  cords  during  inspiration,  expiration  and  phonation 
are  observed.  Fig.  10  shows  the  position  of  the  vocal  cords  in  four 
characteristic  palsies. 


•      CRANIAL  NERVES  41 

Examination  of  tJw  Speech. — A  complete  analysis  of  the  function  of 
language  will  not  be  entered  into  in  this  place,  since  its  modifications 
are  best  considered  under  the  general  head  of  aphasia.  The  chief 
points  of  neurological  interest  to  be  observed  are  whether  the  tongue 
is  protruded  in  the  middle  line,  whether  it  is  freely  movable,  up,  down, 
right,  and  left,  and  can  be  made  to  push  out  both  cheeks.  Careful 
search  should  be  made  for  scars  on  the  tongue,  and  the  presence  of 
a  leukoplakia  on  the  sides  of  the  tongue  or  of  the  mucous  membrane 
of  the  cheeks  should  not  be  overlooked. 

Tremors  of  the  tongue  may  be  very  fine,  involving  the  whole  organ, 
or  may  be  fibrillary  or  coarse  and  irregular.  Considerable  attention 
should  be  devoted  to  the  search  for  tongue  tremors. 

In  testing  ordinary  speech,  certain  test  phrases  are  advisable.  The 
patient  should  be  directed  to  repeat  the  alphabet,  and  the  numbers  up 
to  twenty-five,  and  should  repeat  something  well  known,  such  as 
the  Lord's  Prayer,  or  some  bit  of  poetry,  and  during  the  repetition 
careful  attention  should  be  directed  to  the  enunciation  of  the  individual 
letters,  to  the  presence  of  stumbling  on  words,  of  running  words 
together,  to  the  omissions  of  words,  or  the  omission  of  syllables,  and 
particularly  to  the  repetition  and  the  displacement  of  syllables.  In 
order  to  bring  out  some  of  these  defects,  certain  test  words  are  utilized. 
Among  the  most  valuable  are  the  following:  Truly  Rural;  Third 
Riding  Artillery  Brigade;  Methodist  Episcopal;  National  Intelligencer. 
Naturally  the  type  of  case  will  suggest  certain  defects,  which  can  then 
be  seized  upon  by  the  examiner,  and  the  speech  defects,  when  present, 
brought  out  more  clearly. 

In  stating  the  speech  defects  due  to  laryngeal  loss,  special  attention 
should  be  directed  to  the  presence  of  cough,  of  stridor,  and  to  the  pitch 
of  the  breathing. 

Stuttering  is  a  spasmodic  form  of  speech  disturbance  which  calls  for 
special  mention.  Certain  patients  show  a  very  marked  slowing  of 
speech  (bradylalia),  while  others  show  an  intermittent  enunciation, 
and  still  others  a  peculiar,  monotonous,  semi-sing-song  type  of  enuncia- 
tion known  as  "  scanning  speech."  In  complicated  speech  disturbances 
due  to  involvement  of  the  hypoglossal  nerve  one  obtains  the  so-called 
bulbar  thick  speech :  the  patient  speaks  as  though  he  had  a  hot  potato 
in  his  mouth.  Further,  in  extensive  speech  disturbances  due  to 
coexisting  lesions  in  different  parts  of  the  speech  mechanism  one 
has  other  disturbances  known  as  anarthria,  or  more  particularly  as 
dysarthria,  or  "jumbled  speech."  The  subject  of  aphasic  speech  will 
be  discussed  later. 

The  Tenth  Nerve. — The  study  of  the  heart  action  is  referred  to  the 
chapter  on  the  Vegetative  Nervous  System. 

The  Eleventh  Nerve  or  Spinal  Accessory. — This  nerve  supplies  the 
sternomastoid  and  the  trapezius.  .  Its  functional  capacity  is  tested 
by  the  ability  to  raise  the  shoulders  and  to  turn  the  neck,  pressure 
Being  made  on  the  chin  in  resistance.     There  are  a  number  of  normal 


42 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


displacements  resulting  from  paralysis  of  this  nerve  which  will  be 
discussed  later  under  the  head  of  Paralysis. 


Trajyezius  XI.C2,  i 
Sterno  oleido  mastoid 
XI.  C2-S~ 


Subclavius  Co, 
Deltoid- 
Subscapularis  C5{G), 
Coraco  brachials 
OS, 
Pectoralis  minor_^ 
Latissimus  dorsi 
C6-8~ 


Serratus  magniis 


Superficial  niusoles 
of  the  face  VII 


Infe)  lor  hyoid  region 

XII  pair 

In  ternal  descending 

branch  C2,S 

Platysma  niyoides 
VII+C2 


Pectoralis  major 


^^^     (clavicular) 
-\- Deltoid 

Pectoralis  major 
{sternal) 


Pectoralis  major 
(costal) 


Supinator  brevis_ 
C5,  G,  7 


Intel  nal 
I ectus  " 
Li-i 
Adductoi  uiagnui, 

■^■^    Anterioi  lettm         Addiuto) 
{Red.  fern.)  longus 


•Brachialis  anticus  C5,  6 
Pronator  rad. 

teres 
Ex  tens.  carp, 
rad.  I.  C(5)6,  7 
Supinator 

longus 
Exteiis.  carp, 
rad.  brev. 
'0(5)6,  7 
I'aliHaris 
longus 
'almaris 
"'  brevis 
Flexor 
sublimis 
Ugitorum 
CI,  8  Dl 
Flexor 
longus 
nollicis 


Anterio)  rectus 
(Beet,  fern.) 


Fig.  11. — Radicular  innervation  of  the  muscles  of  the  anterior  regions  of  the  head, 
neck,  trunk,  and  upper  extremities.  On  the  left,  the  superficial  muscular  layer;  on  the 
right,  the  deep  muscular  layer.     (After  Dejerine.)   C,  cervical;  D,  dorsal;  L,  lumbar. 


CRANIAL  NERVES 


43 


Inferior  inaxiUary 
Internal  descending 
branch  cervical  ijlexus 
Hyjmglossal 

N.  to  anuularis 
N.  toscaleni 

N.  of  rectus  capitib 

anticus  major 

C.P.  C'l-i 


Anterior  thui  acic  mt 


Subscapularis 
sup.  et  inf. 
Brachial  plejcub 


Latissimus  dorsi 

B.F. 

Posterior  thoracic 

B.P. 

Musculo  cutaneoub^ 
Brachial  plexus 


Facial  N. 


Maxillary  inferior  N. 

Spinal  accessory  XI 
'Sterna  cleido  mastoid  C.P. 

Sjnnal  accessory  XI 
^to  ti  apezius  C.P. 


Musculo  cutaneou 

and  radial  '^^ 

Brachial  plexus 

Intercostals 
Hypogastric 


Circumflex 
Brachialjilexus 

-Anterior 
thoracic  ext. 
rachial  x>lexus 

-Anterior 
thoracic  int. 
Brachial  plexus 

-Musculo 
cutaneous  B.P, 


3Iedian  B.P. 
Hadial  B.P. 


Obt  urator 
Lumbar  plexus 

Fig.  12. — Peripheral  innervation  of  the  anterior  muscles  of  the  head,  neck,  trunk, 
and  upper  extremities.  To  the  left  the  superficial  muscular  layer;  the  deep  muscular 
layer  to  the  right.  The  same  markings  on  this  and  the  preceding  figure  are  followed 
for  the  facial,  trigeminal,  spinal  accessory,  hypoglossal,  cervical  plexus,  and  inter- 
costal nerves  respectively.  Different  shadings  differentiate  different  innervations  m 
the  brachial  plexus.  (After  Dejerine.)  The  chief  plexuses  are  indicated.  C.P.,  cervical 
plexus;  B.P.,  brachial  plexus;  L.P.,  lumbar  plexus;  S.P.,  sacral  plexus;  D.  in  Fig.  11 
indicates  dorsal  roots;  I.X.  in  Fig.  12,  intercostal  nerves. 


44  METHODS  OF  NEUROLOGICAL  EXAMINATION 

Ninth,  Tenth,  and  Twelfth  Nerves. — The  glossopharyngeal,  vagus, 
and  hypoglossal  nerves  may  be  considered  more  or  less  together  in 
their  testing.  The  glossopharyngeal  is  involved  in  the  function  of 
taste  of  the  posterior  third  of  the  tongue  and  of  the  soft  palate.  It  is 
also  a  nerve  of  common  sensation  for  the  back  of  the  tongue,  part 
of  the  soft  palate,  and  upper  part  of  the  pharynx.  It  supplies  the 
middle  constrictor  of  the  pharynx  and  the  stylopharyngeus  with 
motor  fibers. 

For  further  details  of  the  testing  of  the  other  nerves  see  the 
chapter  on  Cranial  Nerves. 

Upper  Extremities. — A  systematic  examination  of  the  upper 
extremities  is  next  in  order,  the  muscular  apparatus  first  claiming 
attention.  The  two  sides  of  the  body  should  be  examined  system- 
atically.    (See  Figs.  11  to  14.) 

Anomalies  of  structure  should  first  be  noted,  such  as  habit  or  occupa- 
tion positions,  alterations  of  posture,  etc.  Gross  differences  in  the  size 
of  the  bones,  the  wrists,  hands,  etc.,  should  be  measured. 

Atrophy. — This  may  be  determined  by  simple  palpation  and  by 
measurement.  After  natural  differences  in  the  muscular  volume 
are  taken  into  consideration,  striking  variations  should  be  carefully 
measured.  The  circumferences  over  the  biceps  and  just  below  the 
elbow^s  on  the  two  sides  should  be  compared.  Special  individual 
muscular  atrophies  may  be  picked  out  later  by  means  of  electrical 
reaction  tests.  Atrophies  of  the  nails,  skin,  or  hairy  structures  can 
be  recorded  here,  or  under  the  heading  Neurotrophic  Disorders,  which 
appears  later  in  the  questionnaire. 

Hyijertrophy . — This  is  best  brought  out  by  palpation  and  by  measure- 
ment. Due  weight  should  be  given  to  natural  variations — right  and 
left  sides — and  to  the  influence  of  certain  occupations — blacksmiths, 
iron- workers,  etc. 

Hypotonus. — This  is  indicated  by  unusual  flaccidity  of  the  muscu- 
lature and  movements.  Sudden  pronation  or  supination  of  the  arm, 
extension  or  flexion  at  the  shoulder-,  wrist-  or  elbow-joints,  may  show 
sudden  sharp  resistances,  followed  by  marked  flaccidity.  Ability  to 
overextension,  etc.,  is  a  sign  of  hypotonus. 

Spasm. — This  indicates  hypertonus.  When  permanently  present, 
contractures  result.  The  particular  muscles  which  may  show  hyper- 
tonus, or  spasticity  or  contractures,  should  be  recorded.  In  certain 
spastic  conditions  the  hypertonus  may  be  relieved  by  passive  move- 
ments.    Such  should  be  sought  for. 

Muscular  Poiver. — This  is  first  tested  by  having  the  patient  execute 
all  the  chief  movements  of  the  shoulders  and  arms.  The  chief  tests 
are  as  follows : 

Shoulder,  Arm,  Hand,  and  Fingers.— Deltoid.— Hequest  the  patient 
to  raise  the  arms  laterally  to  a  horizontal  position.  Inability  so  to  do 
indicates  deltoid  paralysis. 


UPPER  EXTREMITIES  45 

Pectoral  Muscles. — Stretch  out  the  arms  straight  in  front  and  then 
approximate  the  hands  against  resistance  by  the  examiner,  meanwhile 
watching  both  heads  of  the  pectoral  muscle. 

Latissimus  Dorsi. — Raise  the  arms  laterally  to  a  level,  then,  while 
keeping  them  fully  extended,  bring  the  arms  downward  and  backward, 
as  if  to  make  the  hands  meet  behind  the  sacrum.  The  examiner 
standing  behind  the  patient  resists  the  movement. 

Serratus  Magnus. — Desire  the  patient  to  push  with  his  hands  against 
those  of  the  examiner  or  against  a  solid  object.  If  the  serratus  has 
lost  its  power  the  scapula  will  project  and  the  digitations  of  the  muscle, 
which  ordinarily  should  be  visible,  will  not  be  seen. 

Trapezius. — Ask  the  patient  to  raise  the  shoulders  as  close  to  his 
ears  as  possible  against  the  pressure  of  the  examiner's  hands.  This 
will  demonstrate  the  strength  of  the  upper  part  of  the  trapezius. 
The  middle  and  lower  portions  are  tested  by  desiring  him  to  bring 
the  scapulae  as  close  together  as  possible. 

It  is  hardly  possible  to  detect  paralysis  of  the  levator  anguli  scapulse 
and  rhomboids  unless  the  trapezius  is  also  involved. 

Biceps. — Let  the  patient  flex  his  extended  arm,  his  elbow  resting 
in  the  observer's  left  hand,  while  the  latter's  right  hand,  grasping  the 
wrist  of  the  patient,  offers  the  necessary  resistance.  Also  supinate 
the  hand  against  resistance. 

Triceps. — The  triceps  may  be  tested  as  is  the  biceps,  excepting 
that  the  previously  flexed  arm  is  to  be  extended  against  resistance. 

Supinator  Longus. — Test  as  for  the  biceps,  except  that  the  hand 
should  be  midway  between  supination  and  pronation.  If  the  muscle 
is  paralyzed  it  will  fail  to  become  conspicuous  on  the  radial  side  of  the 
upper  part  of  the  forearm. 

Flexors  of  the  Wrist. — Grasping  the  patient's  hand,  the  palm  being 
upward,  desire  him  to  bend  the  hand  up  toward  his  forearm  against 
resistance. 

Extensors  of  the  Wrist. — The  patient's  hand  being  held  palm  down- 
ward, he  is  required  to  bend  it  backward  against  resistance.  Moderate 
weakness  of  the  extensors  of  the  wrist  may  be  manifested  by  asking 
him  to  squeeze  the  examiner's  hand,  in  which  case  the  wrist  will  become 
involuntarily  flexed,  the  weakened  extensors  being  unable  to  counteract 
the  flexors.  Marked  or  complete  paralysis  of  the  extensors  is  wrist- 
drop. 

Flexors  of  the  Fingers. — Because  of  the  usual  difference  in  the  strength 
of  the  two  hands  the  examiner  should  cross  his  forearms  and  place  his 
right  hand  in  the  right  hand  of  the  patient,  and  vice  versa.  Then  let 
the  patient  squeeze  the  hands.  If  the  observer  keeps  his  own  fingers 
extended  and  bunched  loosely  together  he  will  be  able  to  withstand  a 
very  hearty  grasp  without  discomfort. 

Adchictor  Pollicis. — Ask  the  patient  to  pinch  with  his  thumb  and 
finger  one  of  the  examiner's  fingers. 


46 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


Opponens  Pollicis. — Desire  the  patient  to  approximate  the  ends 
of  the  little  finger  and  the  thumb — while  thus  approximated  the 
examiner  pulls  his  finger  through. 


Temporalis 


Complexus  Cl-8 

Splenius  "{'^'''^H  cS-Sp 
of  neck  i 
Rectus  capitis  anticus 
major  CI 
Levator  anguli  scap  GJ 
Scalenus  viedius  02 
Scalenus  posticus  C5-S 
Scalenus  anticus  d-l 
Rhomboideus 
Ci-5  B.F. 


Infraspinatus  C5-G 
Teres  minor  Co 

Teres  major-. 

C(5)6(7) 

CG-Ji_ 
Latissimus  dorsi 
Brachialis  anticus 
Triceps 
Biceps 


Brachio  radialis 
Ext.  carpi  rad.  brev.~ 
Anconeus  _J22IS 

JSxt.  carpi  rod-  Umg,  (76(7)3 

Ext.  carpi  ulnaris  ^ 
Ext.  comm.  digitorum^. 


Flex.  long,  pollicis 

Ext.  brev.  imllicis 

C6,7 
Cfluteus  maximus 

Abducens  pollicis 
C6,7 


1  Dorsal  interossei 
C8  (Dl) 
1  Lumbricales 
C7-8(D1) 

Biceps 


Stylohyoid  {VII,  IX) 


^^Masseter 
Digastric  (VII,  IX) 
Stylohyoid  WII,  IX) 
MillohyoidW) 
^        Inf.  constrictor  of  pharynx 
lliijioliyo  id  Cl-2         -]  ax,  X,  XI ) 
-Uiniihuoid   Cl-3       I 
Stiinohijoid   Cl-3      r-'-' 
Stei  nutliyroid  Cl-i] 
Longus  colli   C2-8 


Pectoralis  major 


Serratus  magnus 
Sup.  Intercostals 
D5,G,7 
Inf.  Intercostals 


Rectus  abdominis 
Transversalis 
Jjfluteus  minimus 


Sarforiits  L2,  3 
Obturator  ext  L3,  4 

— Rectus  femoris 
Adductor  pollicis 

CSiDl) 
Vastus  externus 


Fig.  13. — Radicular  innervation  of  the  lateral  aspect  of  head,  neck,  trunk,  and  upper 
extremity.  The  platysma  myoides,  sternocleidomastoid,  trapezius,  and  oblique  muscles 
of  the  abdomen,  gluteus  medius,  and  tensor  of  the  fascia  lata  have  been  removed.  The 
shading  and  cross-hatching  follow  the  muscles  and  innervation  as  in  Figs.  11  and  12. 
(After  Dejerine.) 


UPPER  EXTREMITIES 


47 


The  interossei  and  lumbricales  muscles  of  the  hand  flex  the  proximal 
phalanges,  and  extend  the  middle  and  terminal  phalanges.   The  dorsal 


Inferior  maxillaru  I 


Post,  branch 
cervical  nerves  Ci-njj 
N.  Rectus  anticus 
and  longus  collis 
C.P. 
N.  Scaleni  C.P. 
N.  levat.  anouli  scap. 
Cervical  plex 
N.  rhomb. and 
lev.  ang.  scup. 
B.P.  Cl-5 

N.  subscapulu)  is 
Brachial  plex  ui> 
Circumflex  B.P 
CiiLuinflt  L  - 
Brachial  I'h  t  as 


Lower  subscapular 
B.P. 
Long  subscapular 
Brachial  plexus 


Musculo  cutaneous 
radial 


Inferior  ma.villary  V 

Stylohyoid  (  VII 

Posterior  digastric  [  IX 

Inferior  dental  V 
Pharyngeal  IX,  X,  XI 
Longus  colli  C.P. 

Int.  deso.  branch  C.P.  C2,ii 
and  Hypoglossus  XII 

Anterior 
thoracic 
Brach  ial  plexus 


I'li^t. thoracic 
lii  achial  pilex  as 


Musculo  cutaneous 
Brachial  plexus 


Ant.  thoracic  int. 


Intercostal  and 
Hyjjogastric  5-12 


Intercostal,  genito 
-'crural  and 
hypogastric. 
Lumbar  plexus 


I        '  11  Superior 

/I       I  //    gluteal      , 

/j   '     /  /Jhucral  plexus 

Crural 
Lumbar  plexus 
\H     Obiurator 
Lumbar  xile.vus 


C>  ui  al 
Lumbar  2>lexus 


Sciatic 
Sacral  pleafus 

Fig.  14. — Peripheral  innervation  of  the  lateral  aspect  of  the  head,  neck,  trunk,  and 
upper  extremity.  Markings  and  cross-hatchings,  as  in  previous  figure,  to  indicate  the 
various  innervations.  Figures  C.D.L.P.,  C.P.,  B.P.,  L.P.,  and  S.P.  as  in  previous 
diagrams.     (After  Dejerine.) 


interossei  adduct,  the  palmar  adduct,  the  fingers  from  and  toward 
a  longitudinal  line  drawn  through  the  center  of  the  middle  finger. 


48  METHODS  OF  NEUROLOGICAL  EXAMINATION 

Test  by  making  the  patient  separate  and  approximate  the  fingers, 
and  flex  the  proximal  phalanges,  keeping  the  middle  and  terminal 
phalanges  extended.     Paralysis  of  these  muscles  causes  "claw-hand." 

The  strength  of  the  hand  grasp  is  well  tested  by  the  dynamometer. 
Readings  of  three  tests  for  each  hand  should  be  recorded.  Dynamo- 
meter records  are  available  for  definite  comparisons.  Further,  the 
dynamometer  is  useful  by  many  repeated  readings  (20)  for  obtaining 
an  idea  of  the  fatiguability  of  muscles  (neurasthenia,  myasthenia, 
alterations  in  attention,  etc.).  Weiler  has  constructed  a  useful 
dynamometer  with  a  graphic  register. 

The  Muscles  of  the  Trunk. — The  erector  muscles  of  the  spine  are 
examined  by  causing  the  patient  to  lie  face  downward  and  asking  him 
to  raise  the  head  and  shoulders  without  assistance  from  the  hands. 
Unless  paralyzed  the  erectors  become  clearly  visible  during  the  attempt. 
The  abdominal  muscles  are  tested  in  a  similar  manner,  except  that  the 
patient  lies  in  the  dorsal  position  while  making  an  effort  to  raise  the 
head. 

The  Lower  Extremities. —  The  muscles  of  the  lower  extremities  are  best 
tested  with  the  patient  in  the  lying  position  (see  Figs.  11  to  16) : 

Flexors  of  the  Thigh. — The  patient  lying  upon  his  back,  ask  him 
to  raise  the  leg  from  the  bed,  against  resistance,  the  knee  being  kept 
straight.  This  determines  the  strength  mainly  of  the  ileopsoas, 
partly  of  the  quadriceps. 

Extensors  of  Thigh. — The  leg  being  kept  straight  and  the  patient 
lying  upon  his  back,  raise  the  foot  and  ask  him  to  bring  it  down  upon 
the  bed  against  resistance.  This  determines  the  strength  of  the 
gluteus  maximus  and  partly  of  the  hamstring  muscles. 

Abductors  of  Thigh. — With  the  leg  across  the  middle  line  ask  the 
patient  to  carry  it  toward  the  outer  side  against  resistance,  thus  testing 
mainly  the  gluteus  medius. 

Inrotators  of  Thigh. — ^With  the  patient  on  his  back,  flex  the  knee  to  a 
right  angle,  grasp  the  foot,  and  oppose  resistance  while  he  inrotates 
the  thigh,  testing  mainly  the  gluteus  minimus. 

Outrotators  of  the  Thigh. — Similarly  test  the  power  of  outrotation, 
thus  determining  the  condition  of  the  obturators,  pyriformis,  gemelli, 
and  quadratus  femoris. 

Flexors  of  the  Knee. — The  patient  lying  upon  his  back,  desire  him 
to  bend  the  knee  while  the  examiner  resists  the  movement  by  grasping 
the  ankle,  thus  ascertaining  the  power  of  the  biceps,  semimembranosus, 
and  semitendinosus. 

Extensors  of  the  Knee.- — With  the  patient  in  the  dorsal  position, 
flex  the  knee,  and  by  pressure  on  the  sole  of  the  foot  resist  his  endeavor 
to  extend  the  knee.  The  quadriceps  femoris  is  the  principal  muscle 
concerned. 

Plantar  Flexors  (Extensors)  of  the  Foot. — With  the  leg  straight, 
resist,  by  pressure  upon  the  sole  of  the  foot,  the  patient's  endeavor 
to  bring  the  tarsus  in  a  line  with  the  leg,  thus  testing  the  gastroc- 


REFLEXES 


49 


nemius,  soleiis,  peroneus  longus  and  brevis.  Have  patient  stand 
on  toes. 

Dorsifledors  of  the  Foot. — With  the  leg  straight,  resist  the  patient's 
attempt  to  bend  up  the  foot,  thus  testing  the  tibiaHs  anticus  and  the 
peroneus  tertius.  Marked  paralysis  of  these  muscles  causes  "foot 
drop."  Have  patient  stand  on  the  heels. 

Muscles  of  the  Foot. — The  flexors,  extensors,  interossei,  and  lumbricales 
of  the  toes  are  examined  in  a  similar  manner  to  those  of  the  fingers. 
There  is  a  sort  of  claw-foot  analogous  to  the  claw-hand. 

Reflexes. — Superficial  and  Deep. — These  are  then  taken  up.  Those 
of  the  cranial  nerves  have  been  considered.  The  important  reflexes 
of  the  upper  extremities  are: 

Elboiv  or  Triceps  Jerk. — This  is  best  tested  by  supporting  the  patient's 
arm  at  the  elbow,  allowing  the  forearm  to  hang  flaccidly  or  putting 
it  over  the  edge  of  a  chair.  The  stroke  is  made  just  above  the  ole- 
cranon, and  the  reaction  consists  in  an  extension  of  the  forearm  due  to 
contraction  of  the  triceps  muscle. 

Radius  Periosteal  Reflex. — The  radius  periosteal  reflex  consists  in  a 
slight  flexion  of  the  arm  on  the  forearm  when  the  radius  is  tapped  just 
three  or  four  inches  above  the  external  condyle. 

Supinator  Jerk. — ^The  supinator  jerk  is  obtained  by  striking  the 
muscle  about  midway  between  the  elbow  and  wrist,  the  arm  being 
supported  at  the  wrist.  It  consists  in  a  slight  extension  of  the  pendant 
wrist. 


Reflex. 

Biceps. 
Triceps. 
Supinator  longus. 

Wrist. 


Method  of  eliciting. 

Tap  biceps  tendon. 
Tap  triceps  tendon. 
Tap  radial  styloid. 


Response. 

Biceps  contracts. 
Triceps  contracts. 
Supinator  longus  con- 
tracts. 


Carpometacarpal.     Tap  back  of  wrist. 


Tap  flexor  tendons  at  wrist.     Fingers  are  flexed. 


Segment. 

C  5  and  C  6. 
C  5,  6  and  7. 
C  5  and  C  6. 

C  6  to  C  8. 


Fingers  are  extended.       C  6  to  D  1. 


Jacobsohn's  radius  reflex  consists  in  a  slight  flexion  of  the  fingers, 
particularly  of  the  terminal  phalanges  when  the  radius  of  the  extended 
outstretched  hand  supported  by  the  observer's  hand  is  suddenly  tapped 
with  a  hammer. 

The  superficial  reflexes  of  the  trunk  are  next  in  order.  Both  sides 
should  always  be  tested  and  recorded  o  if  absent,  +  if  present; 
R  =  L  or  R>  L,  R  <  L,  or  R,  o,  L+,  or  vice  versa,  as  the  case  may  be. 

Epigastric. 
Abdominal. 


Cremasteric. 
Gluteal. 

Bulbocavernosus. 

Superficial  anal. 


Stroke     downward     from 

nipple. 
Stroke   down   from   costal 

margin. 
Stroke  inner  side  of  thigh. 
Stroke  skin  over  buttocks. 

Pinch     dorsum     of     glans 

penis. 
Prick  skin  of  perineum. 


Epigastrium  dimples, 
muscles 


Abdominal 
contract. 

Testicle  is  pulled  up. 

Gluteal  muscles  con- 
tract. 

Compressor    urethras 
contract. 

External       sphincter 
contracts. 


D  7  to  D  9. 
D  11  to  L  2. 


L  1  to  L  2. 
L  4  to  L  5. 


S  3  to  S  4. 
S  5  and  conus. 


50 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


Superior  gluteal 
P. 


Gluteus  maximus 


Crural  N. 
Lumbar  plexus 


Crural  N. 
Lumbar  plexus 


Crural  N. 
Lumbar  plexus 


Peroneus  longus 


Peroneus  tertiub 
Ext.  bvev.  digit. 

Abductor  minim  i  11/ 
digiti 


Fig.  15.— Radicular  (R)  (to  left)  and  peripheral  (P)  (to  right)  innervations  of  the 
muscles  of  the  external  side  of  the  lower  extremity.  Letters  and  abbreviations  as  in 
preceding  figures.     (After  Dejerine.) 


REFLEXES  OF  THE  LOWER  EXTREMITIES 


51 


Reflexes  of  the  Lower  Extremities. — The  knee-jerk  (Erb-Westphal's 
sign)  is  one  of  the  most  famihar.     The  knee-jerk  may  be  tested  in  a 


Pyrami 
dalis  N.  S. 

Obturator 
Internus  S.P.- 
Isohionoccygeus^ 
Genital  Pleaius 

Jiecium  and  Anus  Cf.F^ 

Hemorrhoidal-pl.  G.P- 

VaginaG.P. 

Obturator. 


Crvral 
Lumbar  plexus 


L  ii  )5,S1(2) 

Oljt-iirator  int. 
Pyramidalis 
Ischiococcygeus 
Levator  ani  S3-i 

.jSphincter  ani 
S3-i 


Ibchio-cavemosiis  i  „., 
Bulbo-cavernosus  ] 


Gluteus  maximus 
'Adductor  magnus 
Adductor  longus 

Semi  tendinosus 
~~Seini  membranosus 

-Biceps    L(i)5,si 
~~  Gracilis 


'^-Gastrocnemius 


Soleus 

■Flex.  long.  dig. 

L5,  Sl-2 

Tibialis  posticus 

Lo,  Sl(2) 

Flex.  long,  hall- 
Lo,  Sl-2 


Fig.  16. — Radicular  (R)  (to  right)  and  peripheral  (P)  (to  left)  innervations  of  the 
muscles  of  the  internal  side  of  the  lower  extremities.  (After  Dejerine.)  G.P.,  genital 
plexus. 

variety  of  ways.     One  of  the  best  is  to  have  the  patient  sit  upon  a 
table,  which  permits  the  limbs  to  hang  freely,  then  telling  him  to  look 


52  METHODS  OF  NEUROLOGICAL  EXAMINATION 

at  the  ceiling,  or  diverting  his  attention,  the  tendon  just  below  the 
patellar  is  tapped  or  the  patient  is  directed  to  cross  his  leg  on  the  knee. 
Exaggerated,  active,  normal,  sluggish,  or  absent  responses  should  be 
recorded.  Another  method  is  to  have  the  patient  sitting,  and  the  feet 
upon  the  floor,  but  the  legs  comfortably  extended.  The  tendon  is  then 
tapped.     Here  a  simultaneous  tapping  of  both  tendons  may  be  tried. 

In  certain  patients  the  attention  must  be  diverted,  otherwise  he 
holds  the  leg  rigid.  This  destroys  the  reflex.  One  can  have  the  patient 
repeat  the  Lord's  Prayer,  or  have  him  do  small  sums  in  arithmetic, 
or  converse  with  an  assistant  to  divert  his  attention  from  the  testing. 

Jendrassik  thought  of  the  expedient  of  utilising  a  forced  muscular 
act  in  the  upper  extremity  to  reinforce  the  knee-jerk.  This  may  be 
carried  out  by  having  the  patient  make  hard  fists  at  a  given  signal, 
when  the  tendon  is  tapped,  or  by  having  him  grasp  his  hands  and  pull 
at  the  given  signal.  By  reinforcement  a  very  weak  knee-jerk  may  be 
made  very  evident. 

Achilles  Jerk. — This  is  best  tested  by  having  the  patient  kneel  upon 
a  chair,  the  foot  being  just  free  of  the  edge.  The  Achilles  tendon  is 
then  tapped,  and  there  results  a  dorsal  flexion  of  the  entire  foot. 

For  patients  in  bed,  the  leg  should  be  everted,  slightly  flexed, 
and  the  foot  extended  to  put  the  tendon  on  slight  tension.  One  person 
in  a  hundred  has  no  knee-jerk. 

Ankle-clonus. — To  elicit  ankle-clonus  requires  some  care.  It  is  best 
obtained  by  supporting  the  patient's  leg  along  the  under  side,  the 
patient  cooperating  by  thorough  relaxation,  then  the  free  hand  grasps 
the  foot,  and  makes  a  sudden  upward,  dorsal  flexion,  holding  the 
foot  fairly  firmly  flexed  at  the  end  of  the  movement  when  a  series 
of  clonic  extensions  and  flexions  take  place.  The  leg  should  be  slightly 
everted,  and  the  knee  somewhat  flexed.  A  false  clonus  consists  in  half 
a  dozen  flexions  and  extensions;  true  clonus  continues  for  some  time. 

Plantar  Reflex. — By  stroking  the  sole  of  the  foot,  either  at  its 
external  or  internal  border,  a  quick  plantar  flexion  of  all  the  toes 
including  the  great  toe  takes  place.  This  is  normal  plantar  flexion. 
As  many  individuals  are  ticklish,  there  is  frequently  a  sudden  jerking 
of  the  whole  foot,  or  such  a  protective  movement  is  manifested  only 
in  the  tendons  of  the  great  toe.  This  should  be  distinguished  from 
true  dorsal  extension  or  the  Babinski  reflex.^  This  consists  in  the 
comparatively  slow  dorsal  extension  of  the  great  toe  when  the  plantar 
reflex  is  tested  and  at  the  same  time  there  is  a  slight  spreading  apart 
of  the  other  toes. 

If  the  patient  is  in  bed — as  he  should  be  for  testing — a  fully  developed 
Babinski  sign  consists  in  the  slow  dorsal  raising  of  the  great  toe,  the 
spreading  of  the  other  toes,  a  slight  rotation  of  the  thigh  on  the  hip, 

1  The  term  dorsal  extension  is  here  meant  extension  of  the  toes.  By  Babinski  the 
term  plantar  extension  was  used.  We  are  using  the  words  in  the  ordinary  sense,  dis- 
regarding the  fact  that  by  some  palmar  extension  is  made  synonymous  with  dorsal 
flexion,  and  plantar  flexion  with  dorsal  extension. 


REFLEXES  OF  THE  LOWER  EXTREMITIES  53 

and  a  contraction  of  the  fascia  lata  of  the  thigh.  In  order  to  develop 
the  whole  test  the  feet  should  be  warm,  the  thigh  slightly  rotated 
externally,  the  knee  slightly  bent,  and  the  stroke  made  either  on  the 
outer  or  inner  border  of  the  sole  by  either  a  fairly  sharp  instrument, 


Fig.  17. — Extension  of  the  great  toe  on  irritating  the  sole.     The  Babinski  plantar 
extension  phenomenon. 

the  finger  nail,  or  a  blunt-pointed  instrument.  As  there  is  great  varia- 
tion in  the  thickness  of  the  skin  of  the  soles  of  the  feet,  the  various  ways 
of  bringing  out  a  Babinski  phenomenon  should  be  tried  in  each  case. 
Particular  attention  should  be  directed  to  the  dangers  of  confusing 


Fig.    is. — The   Chaddock  modification  of   the   Babinski,    causing   great   toe   extension 
on  stroking  beneath  the  external  malleolus.      (Chaddock.) 

the  protective,  pulling  away  motion  on  tickling,  which  causes  a  marked, 

quick  dorsal  raising  of  the  great  toe,  from  a  true  Babinski  phenomenon. 

Careful   record   should   be  made   of  the   irregularities  in  plantar 

response.     In  some  patients  there  will  be  plantar  flexion  of  the  small 


54 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


toes  but  no  reaction  of  the  big  toe.  At  times  this  may  be  as  significant 
as  a  true  dorsal  extension.  It  is  to  be  borne  in  mind  that  dorsal  rais- 
ing of  the  great  toe  is  normal  in  infants  and  children  up  to  the  age 
of  successful  walking. 

A  number  of  closely  related  reflexes  have  been  described,  having 
much  the  same  significance  as  the  Babinski  sign,  but  being  less  constant, 
and  at  times  contradictory.     These  are : 

Striimpell  Reflex. — ^This  follows  forceful  pressure  over  the  anterior 
tibial  region  with  a  resultant  dorsal  extension  of  the  great  toe.  It  is 
found  in  a  number  of  conditions  other  than  those  of  functional  dis- 
turbance of  the  pyramidal  tracts. 

Oppenheim  Reflex. — Here  the  inner  surface  of  the  leg  is  sharply 
and  deeply  stroked  by  the  thumb  from  the  middle  to  the  ankle  behind 
the  malleolus.     It  brings  out  a  great  toe  dorsal  extension. 

The  Paradoxical  Reflex. — Called  by  a  variety  of  names,  as  described 
by  Gordon,  it  consists  in  a  dorsal  extension  of  the  great  toe  following 
the  grasping  of  the  deep  muscles  of  the  calf  and  making  a  forceful 
indention  along  their  external  border. 

Mendel- Bechterew. — This  reflex  consists  of  the  dorsal  extension  of 
the  toes,  especially  the  second  and  fifth,  when  the  dorsum  of  the  foot 
is  tapped  about  at  the  base  of  the  middle  toes.  In  reflex  irritability 
this  reflex  is  augmented.  If  plantar  flexion  takes  place  the  authors 
regard  it  as  a  sign  of  organic  affection. 

Chaddock. — This  produces  a  toe  extension  by  stroking  the  side  of  the 
ankle  (Fig.  18). 


Reflex. 

Method  of  obtaining. 

Result. 

Location. 

Knee. 

Tap  patellar  tendon. 

Leg  extended. 

L3  and  L4. 

Achilles. 

Tap  tendo  Achillis. 

Foot  plantar  flexed. 

S,  S4. 

Ankle  clonus. 

Sudden  dorsi  flexion  foot. 

Quick    up     and     down 

Pyramidal  tracts 

movements. 

(L3,  S2). 

Plantar. 

Stroke  soles  of  feet. 

Plantar  flexion  all  toes. 

L3,  S2. 

Babinski. 

Stroke  soles  of  feet. 

Great  toe  dorsal  exten- 

Pyramidal tracts 

sion. 

(L3.  S2). 

Oppenheim. 

Stroke  inner  side  of  calf. 

Dorsal   extension  great 

Pyramidal  tracts 

toe. 

(L3,  S2). 

Paradoxical. 

Deep  pressure  in  calf. 

Dorsal   extension  great 

Pyramidal  tracts 

toe. 

(L3,  S2). 

Tremors. — The  tremors  of  the  upper  extremities  alone  claim  atten- 
tion. Those  of  the  head  may  be  fine  and  oscillatory,  nodding,  rhyth- 
mical, or  jerky.     The  rapidity  of  the  tremors  should  be  noted. 

Tremors  of  the  hand  and  wrist  should  first  be  tested  with  the 
pendant  hand  supported  at  the  wrist.  Then  with  hands  extended  and 
fingers  wide  apart  static  tremors  come  out.  Or  with  the  hands  in 
motion  locomotor  tremors  become  evident. 

Static  tremors  are  either  fine  and  rapid  (eight  to  twelve  per  second), 
or  coarse  and  slow  (four  to  six  per  second).  They  may  be  irregular. 
When  involving  the  whole  arm  one  speaks  of  movements  rather  than 
tremors. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM  55 

Locomotor  tremors  are  in  reality  ataxias.  They  are  brought  out 
best  by  having  the  patient  bring  his  index  fingers  from  any  position 
slowly  to  the  end  of  the  nose,  first,  with  eyes  open,  then  with  eyes 
closed,  test  both  sides  (Finger-nose  Test — F.  N.  T.),  or  the  index  fingers 
should  be  brought  to  touch  each  other  (Finger-finger  Test — F.  F.  T.). 
Here  coarse  irregular  movements  (ataxias)  may  be  brought  out.  The 
patient's  finger  may  overshoot  the  nose  (asynergia).  The  patient's 
ataxia  increases  markedly  as  the  nose  is  reached  (intention  tremor), 
or  increases  only  just  as  the  object  sought  is  arrived  at. 

Ataxia  in  the  lower  extremities  is  tested  by  the  Knee-heel  Test 
(K.  H.  T.),  the  patient,  on  his  back,  is  directed  to  touch  the  left  knee 
with  the  right  heel,  and  vice  versa. 

Athetoid  movements  are  coarse,  slow,  sinuous,  progressive,  rhyth- 
mical movements  in  the  fingers,  arm,  or  trunk. 

Choreic  movements  are  irregular,  coarse,  or  fine  movements,  non- 
rhythmical  and  non-coordinated — they  are  jerky  movements. 

Associated  movements  are  involuntary  movements  of  the  opposite 
side,  induced  by  a  voluntary  act.  Not  infrequently  they  are  quite 
non-homologous  movements. 

Localized  convulsive  movements  and  Jacksonian  epileptic  move- 
ments consist  of  sudden  convulsive  involuntary  extensions  and  flexions 
without  loss  of  consciousness. 

Tics.— These  are  involuntary,  coordinated  movements  of  psychic 
origin. 

Diadokokinesis. — This  signifies  the  ability  to  perform  alternate 
rapid  coordinated  movements,  of  antagonistic  muscles.  Certain 
patients  show  a  loss  of  this  ability  (adiadokokinesis) .  The  tests 
most  frequently  applied  are  quick  pronation  and  supination  of  the 
semi-flexed  hand;  piano-playing  movements  or  quick  flexion  and 
extension  of  the  forearm  on  the  arm.  The  term  is  applicable  only 
in  the  absence  of  motor  paresis  or  gross  anesthesia. 

Apraxia. — This,  speaking  generally,  consists  in  the  loss  of  ability 
to  perform  purposeful  movements.  The  tests  are  to  have  patient 
throw  a  kiss,  to  make  a  salute,  to  make  a  beckoning  gesture,  a  threaten- 
ing gesture,  or  to  go  through  an  imaginary  act,  such  as  taking  a  match 
out  of  a  box  and  lighting  it  or  blowing  it  out.  It  is  also  elicited  testing 
the  necessary  movements  to  employ  objects  correctly. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM. 

The  most  important  of  the  tests  of  the  sensory  nervous  system  are 
for:  (1)  light  touch,  (2)  pain,  (3)  thermal  sensations,  and  (4)  deep 
sensibility.  Head  has  suggested  the  terms  epicritic,  protopathic,  and 
deep  sensibility  for  the  three  types  of  sensibility  which  he  maintains 
exist. 


EXPLANATION  OF  PLATE  I. 

The  Pyramidal  Tract  in  its  Corticospinal  and  Cortico- 
nuclear Portions. 

The  corticomedullary  (corticospinal)  Co.M,  is  colored  light  red;  the  corticonuclear 
portion,  Co.N,  darker  red.  The  reticular  substance  {S.R.)  of  the  tegmentum  in  yellow 
and  the  motor  nuclei  of  the  cranial  nerves  orange.  The  corticopontine  portions  and 
the  cerebellar  paths  of  the  tegmentum  are  omitted. 

Abbreviations:  Aq,  aqueduct  of  Sylvius;  Cia,  anterior  segment;  dig),  knee;  Cip, 
posterior  segment;  CirP,  retrolenticular  segment  of  the  internal  capsule;  CoM,  cortico- 
spinal system  (pink) ;  CON,  corticonuclear  system  (red)  of  the  pyramidal  tract,  VP; 
Flp,  posterior  longitudinal  fasciculus;  FPba,  FPop,  anterior  and  posterior  pontine 
fibers;  FPyc,  crossed  pyramidal  tract;  FPyd,  direct  pyramidal  tract; /Py/i,  homolateral 
pyramidal  fibers;  fab,  medullary  aberrant  fibers;  fap,  pontine  aberrant  fibers;  fabp, 
medullary,  pontine  aberrant  fibers;  fasth,  subthalamic  or  superior  pontine  aberrant 
fibers;  fcnc,  fend,  crossed  and  direct  cervical  corticonuclear  fibers;  Ln,  locus  niger; 
NC,  caudate  nucleus;  NC,  tail  of  caudate  nucleus;  NCp,  nuclei  of  posterior  columns 
(Goll  and  Burdach) ;  NLi,  NL2,  NL3,  the  three  segments  of  the  lenticular  nucleus;  Np, 
pontine  nuclei;  PLp,  deep  pes  lemniscus;  PLs,  superficial  pes  lemniscus  or  aberrant 
pontine  fibers;  a,  b,  c,  the  three  modes  by  which  the  pontine  aberrant  fibers  enter  the 
third  nerve  nuclei:  Pul,  pulvinar;  Qa,  Qp,  anterior  and  posterior  corpora  quadrigemina; 
RgRm,  region  of  the  median  lemniscus;  Rni,  the  median  lemniscus;  Sgc,  subependymal 
gray  substance;  SR,  reticular  formation,  colored  yellow;  Th,  thalamus;  Vi,  fourth 
ventricle:  VP,  pyramidal  tract;  III,  IV,  nuclei  and  root  fibers  of  the  oculomotorius 
and  trochlearis  nerves;  Vm,  nucleus  and  root  fibers  of  the  trigeminus  (masticators); 
VII,  IX,  XI,  XII,  nuclei  and  root  fibers  of  the  facial  (VII),  glossopharyngeal  {IX), 
spinal  accessory  {XI),  and  hypoglossal  {XII),  nerves;  Xa,  anterior  root  of  the  spinal 
vagus;  X-XI,  root  fibers  of  the  spinal  vagus. 

The  encephalic  trunk  and  its  three  great  divisions:  Cerebral  peduncles,  P;  the  pons 
PO,  the  medulla,  B,  are  shown  in  sagittal  section  with  its  connections  with  the  internal 
capsule  {Cia,  Cig,  Cip,  Cirl)  and  the  thalamus  {Th)  in  part;  and  with  the  spinal  cord 
{M)  for  the  other  part.  The  anterior  segment  contains  the  pyramidal  tract  {V.P)  and 
is  made  up  from  above  below  of  the  foot  composed  of  the  fibers  passing  through  the 
knee  {Cig)  and  the  posterior  segment  {Cip)  of  the  internal  capsule,  the  anterior  segment 
of  the  pons  with  the  pontine  nuclei  {Np)  and  the  anterior  {FPoa)  ancfposterior  {FPop) 
pontine  fibers  and  the  anterior  pyramid  of  the  medulla  {Py) ;  and  the  posterior  segment 
or  tegmentum,  is  separated  above  by  the  locus  niger  {LN),  below  it  abuts  the  posterior 
pontine  fibers  {FPop.),  and  the  pyramid  of  the  medulla  {Py). 

The  tegmentum  is  made  up  of  (1)  a  gray  substance  the  reticular  formation  {S.R) — 
colored  yellow — which  extends  from  the  subthalamic  region  {RsTh)  to  the  lateral  columns 
of  the  cord  and  contains  the  motor  nuclei  of  the  cranial  nerves — colored  orange — disposed 
in  two  longitudinal  columns;  the  anterior  column  includes  the  motor  nuclei  of  the  trigem- 
inus {Vm),  the  facial  {VII),  the  nucleus  ambiguus  or  the  anterior  spinal  vagus  nucleus, 
{Xa) ;  the  posterior  column  includes  the  nuclei  of  the  {III)  and  {IV)  pair,  the  nuclei 
of  the  ( VI) ,  the  long  nucleus  of  the  hypoglossal  {XII)  and  the  long  inferior  nuclei  or 
spinal  accessory  {XI) ;  (2)  of  the  longitudinal  fibers  of  which  a  part  group  themselves  in 
fascicles  more  or  less  compact  to  form  the  posterior  longitudinal  fasciculus  {Flp)  and  the 
median  lemniscus  {Rm).  The  median  lemniscus,  represents  an  important  sensory  path- 
way which  terminates  in  the  thalamus  {Th)  and  takes  part  of  its  origin  from  the  posterior 
column  nuclei  (Goll  and  Burdach).  The  posterior  longitudinal  fasciculus  consists  of 
an  important  association  fiber  system  between  the  nuclei  of  the  oculomotorius,  the  spinal 
(56) 


PLATE  I 


Pyramidal  1  Cortico-nuclear  fibers 
trad       1  cortico-spinal  fibers 


III,  Vl.  XI        r      Deep  pes  lemniscus 
Dorsal  aberrant  } 

pons  fibers      (Superficial  pes  lemniscus 


Fasciculus 
ong    posticus 


t  pyramidal  fibers 
XI,  ClI-IV  aberrant  medullary  fibers' 
Homolateral  pyramidal  fibers 


XII,  XI,  CII-IV  crossed 
cortical  nuclear  fibers 

Crossed  pyramidal 
tract 


nuclei,  the  facial,  to  serve  the  important  functions  of  lateral  movements  of  the  head, 
eyes,  and  trunk  for  maintaining  the  static  equilibrium  of  the  body.     (See  Plate  VIII.) 

Pyramidal  Tract. — The  corticospinal  portion  (CoM)  of  the  pyramidal  tract  (pink) 
takes  its  origin  in  the  ascending  frontal  convolutions  (Fa),  descends  in  the  posterior 
segment  of  the  internal  capsule  (Cip)  and  in  the  anterior  portion  of  the  cerebral  peduncle. 
The  corticonuclear  (CON)  portion  (red)  arises  from  the  Rolandic  operculum  (OpR) 
at  the  foot  of  the  ascending  frontal  convolution  and  the  second  frontal,  descends  through 
the  knee  (Cig)  of  the  internal  capsule  and  along  the  lenticular  border  of  the  posterior 
limb  of  the  internal  capsule  (Cip)  then  to  the  cerebral  peduncle,  where  it  divides.  One 
portion  of  the  corticonuclear  contingent  follows  the  course  of  the  corticospinal  fibers 
to  the  anterior  segments  of  the  pons  and  the  anterior  pyramidal  region  of  the  medulla. 
Another  portion,  the  system  of  the  aberrant  fibers  of  the  pyra?nidal  tract,  break  up  at 
different  levels,  penetrating  the  tegmentum,  descending  in  the  median  lemniscus  (Rm) 
and  the  interolivary  portion  of  the  medulla  to  make  up  the  fibers  entering  the  motor 
nuclei  of  the  cranial  nerves. 

In  the  medulla  portions  of  the  corticonuclear  fibers  fuse  more  or  less  with  the  aberrant 
fibers,  pass  by  the  inferior  hypoglossal  and  spinal  accessory,  and  behave  like  the  cortico- 
spinal fibers.  One  part  descends  in  the  direct  pyramidal  (FPyd),  another  traverses 
the  middle  line  with  the  crossed  pyramidal  (FPyc),  a  third  descends  as  the  homolateral 
pyramidal  (FPyh)  and  the  homolateral  lateral  cord.  All  of  these  fibers  end  finally  in 
the  motor  nuclei  of  the  anterior  horns  of  the  cord,  functioning  the  movements  of  rotation, 
flexion,  and  bending  of  the  head  on  the  neck  (Cu  to  Civ) . 

The  aberrant  fibers  of  the  pyramidal  tract  which  detach  themselves  from  the  cortico- 
nuclear contingent  are:  (1)  some  inconstant  fibers  in  the  subthalamic  region,  the  sub- 
thalamic aberrant  fibers  (fasth)  destined  for  the  oculomotor  nuclei  (Ilia)  and  to  the 
anterior  corpora  quadrigemina.  (2)  In  the  region  of  the  cerebral  peduncles  the  aberrant 
pyramidal  fibers  proper  deep  pes  lemnisci  (PLp),  and  superficial  (PLs)  which  leave  the 
nuclei  of  the  oculomotor  nerves  (b  and  e),  then  descend  in  the  median  lemniscus  (Rm) 
and  contain  fibers  for  the  (VI)  and  (XI)  nuclei-cortical  oculo-rotary  and  cortical  cephalo- 
rotary  fibers.  (3)  In  the  pons  region,  the  pontine  aberrant  fibers  (fap)  which  reinforce 
the  preceding  in  the  median  lemniscus  to  enter  the  motor  nuclei  of  the  trigeminus  (Vm) 
the  hypoglossal  (XII)  and  the  spinal  vagus  (Xa).  (4)  In  the  neighborhood  of  the 
ponto-medullary  sulcus,  the  meduUopontine  aberrant  fibers,  which  are  constant  and 
well  developed,  reinforcing  the  preceding  and  destined  to  the  nuclei  of  the  facial  (VII), 
the  mixed  nuclei  of  the  glossopharyngeal  (IX)  and  the  spinal  vagus  (Xa),  and  the 
hypoglossus  nucleus  (XII).  (5)  In  the  medulla,  medullary  aberrant  fibers,  homologues 
of  the  homolateral  pyramidal  fibers,  in  constant  fibers  which  descend  the  length  of  the 
periphery  of  the  homolateral  antero-lateral  cord  and  terminate  in  the  superior  cervical 
cord  nuclei  of  the  rotators  and  flexors  of  the  head  and  of  the  neck. 

These  aberrant  fibers  present  great  individual  variation  in  their  development — as 
seen  in  the  different  degeneration  pictures  studied  by  the  Marchi  method.  Their  chief 
characteristic  is  their  descending  pathways  in  the  tegmentum,  particularly  in  the  median 
lemniscus  and  the  anterolivary  space  in  the  medulla.  This  disposition  explains  why  these 
fibers  can  be  affected  or  spared  by  peduncular  lesions,  according  to  the  site  above  or 
below,  or  whether  it  involves  the  anterior  or  posterior  (tegmental)  regions.  The  mode 
of  termination  of  these  corticonuclear  fibers,  possibly  direct,  possibly  by  intercalated 
neurones,  in  the  motor  nuclei  of  the  cranial  nerves  has  not  yet  been  elucidated  (Dejerine). 

(57) 


58 


METHODS  OF  NEUROLOGICAL  EXAMINATION 


1.  Epicritic  sensibility  is  that  which  recognizes  light  touch,  dis- 
tinguishes small  differences  between  the  points  of  a  compass,  and 
recognizes  small  variations  in  the  temperature  of  objects. 


Fig.  19. — Illustrating  the  segmental  spinal  sensory  areas.    Front  view. 

2.  Protopathic  sensibility  recognizes  pain  and  extremes  of  heat 
and  cold. 

3.  Deep   sensibility  recognizes   deep   pain   and   muscle   and  joint 
sense.     Bony  sensibility  is  included  here. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM 


.59 


To  test  epicritic  sensibility  one  first  tries  the  method  of  light  touch. 
Touching  the  skin  with  the  end  of  the  finger  is  not  a  light  touch  test. 
It  is  a  test  for  deep  sensibility.     Such  a  test  is  too  coarse.     Either  a 


Z)3- 


FiG.  20. — Illustrating  the  segmental  spinal  sensory  areas.    Rear  view.    These  or  similar 
sensory  charts  are  useful  in  plotting  sensory  changes. 

wisp  of  cotton-wool  should  be  used  or  a  fine  camel's-hair  brush.  For 
most  purposes  the  cotton-wool  is  to  be  preferred.  In  accurate  testing, 
special  esthesiometers  are  indispensable.  Those  mostly  in  use  are 
Van  Frey's  hairs  or  Franz's  simple  esthesiometer. 


60  METHODS  OF  NEUROLOGICAL  EXAMINATION 

The  patient's  body  is  systematically  explored.  He  is  asked  to 
say  "Yes"  every  time  he  is  touched,  and  then  asked  to  localize  the 
spot  touched.  The  testing  should  be  made  with  the  patient  first  lying 
down,  and  due  attention  should  be  given  to  the  character  (thickness, 
etc.)  of  the  individual  patient's  skin  in  drawing  conclusions  from  the 
tests.  In  going  over  the  extremities,  care  should  be  taken  to  circle 
the  limb  wdth  the  touches  as  well  as  going  up  and  down.  It  is  specially 
desirable  to  avoid  suggestive  questions,  such  as.  Do  you  feel  this? 
What  do  you  feel?  etc.  In  certain  cases,  usually  hysterical,  one  will 
get  the  steady  response  "No"  to  each  touch  over  the  so-called  anes- 
thetic area.  This  is  a  highly  suggestive  reaction.  Modifications  of 
light  touch  should  be  charted  and  marked  on  the  skin  with  an  anilin 
pencil.  Such  indications  are  very  useful  as  landmarks  for  localization. 
There  may  be  anesthesia  to  light  touch  or  hyperesthesia,  the  patient 
feeling  very  acutely.  In  all  hairy  parts  the  skin  should  be  shaved  for 
accurate  testing.      (See  Plates  X  and  XI  for  the  pathways  involved.) 

A  Weber  compass  is  useful  for  testing  the  individual  capacity  for 
recognizing  one  or  two  points.  There  is  a  great  variability  in  individ- 
uals and  in  different  regions.  Some  of  the  available  figures  for  the 
minimum  separation  distance  recognized  as  two  points  are  as  follows: 

Tongue 1.5    mm. 

Finger  tips 2.3    mm. 

Lips .       3.4    mm. 

Dorsum  of  fingers 5.6    mm. 

Forearm 9.10  mm. 

Forehead 10  to  15  mm. 

Neck 23  to  30  mm. 

Leg:    back  of  foot 30  to  40  mm. 

Back 50  to  60  mm. 

Arms  and  thigh 70  to  80  mm. 

,  Further  tests  may  be  made  of  epicritic  light  touch  by  placing 
variously  shaped  objects  on  the  skin.  Such  tests  are  very  valuable 
in  special  cases. 

Epicritic  thermal  sensibility.  This  is  most  easily  tested  by  use  of 
the  back  of  the  finger  for  warm,  and  the  metal  head  of  a  percussion 
hammer  for  cool. 

Epicritic  thermal  sensibility  recognizes  differences  as  small  as  two  to 
five  degrees  of  temperature,  while  protopathic  sensibility  is  unable  to 
recognize  differences  between  40°  and  20°  C.  Loss  of  epicritic  sensi- 
bility for  heat,  with  preservation  of  protopathic  thermal  sensibilit}^, 
is  not  uncommon.     The  reverse,  while  rare,  is  occasionally  found. 

In  making  careful  thermal  tests  an  electrical  thermometer,  as 
contrived  by  Mills,  is  useful.  In  ordinary  routine  work  test-tubes 
with  ice  water  and  hot  water  may  be  used,  or  metal  tubes  which 
have  been  plunged  in  cold  or  hot  water  employed. 

It  is  not  sufficient  to  test  patients  for  extremes  of  heat  and  cold 
alone;  minute  differences  should  be  tested  as  well. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM 


61 


Protopathic  Sensibility. — Pain. — This  is  quickest  tested  by  pinching 
the  skin  between  the  nail  and  the  finger.     A  sharp-pointed  pin  with  a 
round  glass  head  is  also  useful.     The  patient  is  asked  to  distinguish 
between  head  and  point.     Absence  of  pain 
yXl  (analgesia)   should  be  carefully    charted    as 

well  as  increased  pain  sensibility  (hyperal- 
gesia). The  limbs  should  always  be  tested 
in  their  circumference  as  well  as  in  their 
length,  care  being  taken  not  to  overlook  thin 
strips  of  analgesia  from  root  lesions.  Hair 
sensibility  should  also  be  tested  by  pulling 
the  hair. 


D5 


'D7 


hSi 


Fig.  21. — Illustrating  the 
segmental  spinal  sensory 
areas.     Side  view. 


Fig.  22. — Illustrating  the  segmental  spinal  sensory 
areas  of  the  lower  extremities. 


Painful  faradic  stimulation  is  at  times  of  value  in  determining  the 
value  of  an  existing  analgesia. 


62  METHODS  OF  NEUBOWOICAL  EXAMINATION 


Ll- 


\K 


Fig.  23. — Cutaneous  reflex  zones  of  hyperalgesia,  showing  their  relations  with  the 
spinal  root  segments  and  their  vegetative  nervous  system  connections.  The  dotted 
areas  are  to  be  referred  to  the  internal  surfaces.     (After  Dejerine.) 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM 


63 


Deep  Sensibility. — ^Here  deep  pressure  pain,  muscle  and  joint  sense 
and  bony  sensibility  are  to  be  tested.  Deep  pressure  with  the  thumb 
and  fingers,  or  a  special  instrument  (baresthesiometer),  is  used.  The 
pressure  should  be  sufficient  to  cause  pain. 

Muscle  and  joint  sense  are  tested  by  first  showing  the  patient  that 
one  moves  the  thumb  and  big  toe  up  or  down — and  then  repeating 
movements  up  or  down  while  the  eyes  of  the  patient  are  closed.  Fur- 
ther, weights  may  be  used  and  the  ability  to  estimate  differences 


Temporal  (,D7) 


Vertical  (,D8) 


Orbital  (.D9,  3 

Nasofrontal  (C  3,4) 
TemiD  or  of  rental  {0  5,6) 


Maxillary  - 
Nasolabial 
Mental 


'arietal  {DO) 


'ccipital  (.DIO) 


Superior  Laryngeal 
Inferior  Laryngeal 


Fig. 


24. — Cutaneous  reflex  zones  of  hyperalgesia  of  the  head,  neck,  and  shoulders  in 
their  relations  to  vegetative  nerve  (somatic)  disturbances.     (After  Dejerine.) 


observed ;  or  the  patient  is  requested  to  imitate  with  one  hand,  a 
definite  position  of  the  other  hand. 

Bojiy  sensibility  is  tested  by  a  tuning  fork  of  low  vibrating  capacity. 

Sensibility  of  the  nerve  trunks  to  direct  pressure  should  then  be 
tested.  In  the  upper  arm  the  brachial  plexus  branches  in  the  neck 
and  under  the  arm  are  palpable  and  along  the  inner  arm  and  elbow- 
joint  one  may  reach  the  median,  radial,  and  ulnar.  Anesthesia  of  the 
ulnar  (Biernacki)  is  frequently  a  tabetic  symptom. 

The  radicular  and  peripheral  sensory  distributions  are  shown  in 
Figs.  11, 12, 13, 14, 15, 16. 


64  METHODS  OF  NEUROLOGICAL  EXAMINATION 

In  the  lower  limb  the  sciatic,  anterior  crural,  cutaneous  femoris, 
tibialis,  and  superficial  peroneus  are  palpable. 

Lasegues  Test. — This  consists  in  flexing  the  extended  leg  on  the 
abdomen,  holding  the  patellar,  when  in  neuritic  processes  a  sharp 
pain  (subpatellar  space)  is  brought  out.  It  is  an  indispensable  test 
in  the  presence  of  suspected  alcoholism. 

The  distribution  of  pain  in  neuralgic  or  neuritic  affections  should 
be  carefully  charted. 

With  the  sensory  examination,  gnostic  and  praxic  tests  should  be 
carried  out. 

Stereognosis  signifies  the  ability  to  recognize  objects  by  touch. 
Astereognosis  is  its  absence.  Objects  should  not  only  be  named  but 
their  qualities  described — shape,  margins,  density,  etc.  A  lump  of 
sugar,  thimble,  match  box,  marble,  knife,  pencil,  scissors,  etc.,  are 
useful  test  objects. 

Apraxia  consists  in  the  loss  of  ability  to  carry  out  a  purposeful 
movement,  not  dependent  on  a  palsy.  The  most  useful  tests  have 
been  referred  to.  The  examiner  is  specially  referred  to  Plates  X, 
XI  for  the  interpretation  of  his  findings  and  their  anatomical 
foundations. 

Vasomotor  and  Trophic  Disturbances. — The  presence  of  dermo- 
graphia,  of  blushing,  of  redness,  or  blanching  of  the  skin  should  be 
looked  for.  Ulcers,  thickness  of  skin,  dryness,  or  other  trophic 
disorders  should  be  charted.  Reflex  hyperalgesias  (referred  pains) 
should  always  be  inquired  for.  Patients  refer  to  them  chiefly  as 
"sore  spots."     (See  Figs.  23  and  24.) 

Scheme  for  Testing  Sensibility. — The  following  scheme  for  testing 
sensibility  is  advised: 

A.  Spontaneous  Sensations:   Pain,  numbness,  tingling,  position  of 

the  limb,  idea  of  the  limb,  hallucinations  or  illusions. 

B.  Loss  of  Sensation: 

1.  Touch. 

(a)  Light    touch,   cotton-wool   on    hairless   and   shaved 

hair-clad  parts;  threshold  with  von  Frey's  hairs. 
(6)  Pressure  touch,  threshold  with  pressure  esthesiometer. 

2.  Localization:  Naming    the    part    touched.     Henri's    or 

Head's  method,  target,  etc. 

3.  Roughness:  Threshold    with    Graham-Brown's    esthesio- 

meter.    Sand-paper   tests,    discrimination   of   relative 
roughness. 

4.  Tickling    and    scraping:  Tickling    on    soles    and    palms. 

Cotton-wool  rubbed  over  hair-clad  parts.     Light  scrap- 
ing with  finger  nails. 

5.  Vibration,  tuning  fork:  Loss  or  diminution  of  sensibility. 

Alteration  in  the  character  of  the  sensation  evoked. 

6.  Compass  points:  Points  simultaneously  applied.     Points 

successively  applied. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM  65 

7.  Pain: 

(a)  Superficial   pain:  pin   prick;   threshold    with   algesi- 

meter;  reaction  to  measured  painful  stimuli. 

(b)  Pressure  pain:  threshold  with  the  algometer;  reaction 

to  painful  pressure. 

8.  Temperature:  Thresholds  for  heat  and  cold.     Effect  of 

adaptation  on  threshold.  Discrimination  of  different 
degrees  of  heat  and  cold.  Affective  reactions:  (a)  to 
extreme  degrees,  (b)  to  warmth. 

9.  Position:  By  imitating  with  the  sound  limb  the  position 

of  the  affected  limb;  by  pointing  with  the  sound  limb; 
measurement  of  defect  by  Horsley's  method. 

10.  Passive  movement:  Appreciation  of  movement.     Recogni- 

tion of  the  directions  of  movement.  Measurement  of 
the  angle  of  the  smallest  movement  which  can  be 
appreciated;  falling  away  of  the  unsupported  limb 
when  the  eyes  are  closed. 

11.  Active  movement:  Imitation  of  movement  by  the  sound 

limb;  ability  to  touch  a  known  spot;  measurement  of 
the  defect  by  Horsley's  method. 

12.  Weight: 

(a)  With  hand  supported:  Recognition  of  differences  in 
weights  applied  successively  to  one  hand.  Appre- 
ciation of  increase  or  decrease  of  weight.  Com- 
parison of  two  weights  placed  one  in  each  hand. 

(6)  With  hand  unsupported:  Comparison  of  two  weights 
placed  one  in  each  hand.  Recognition  of  differ- 
ences in  weights  applied  successively  to  one  hand. 

13.  Size:  Difference;    threshold.     Distinction    of    the    head 

from  the  point  of  the  pin. 

14.  Shape  (two  dimensional). 

15.  Form  (three  dimensional) :  Recognition  of  common  objects 

by  their  form. 

16.  Textures. 

17.  Dominoes:  Ability  to  count  points  by  touch. 

18.  Consistence. 

19.  Testicular  sensibility : 

(a)  Light  pressure. 
(6)  Painful  pressure. 

20.  Sensibility  of  glans  penis  to  measured  prick. 

Status  Corporis. — A  systematic  physical  examination  is  a  sine  qua 
non.  The  main  facts  to  be  noted  in  the  questionnaire  are  the  condition 
of  the  heart,  the  presence  of  murmurs,  the  character  of  the  arteries 
(hard,  tortuous),  blood-pressure,  the  lungs,  presence  of  tumor  in 
abdomen,  enlargement  of  liver,  and  the  condition  of  the  urine,  the 
blood,  and  the  cerebrospinal  fluid.^ 

1  Further  neurological  data  will  be  taken  up  in  the  mental  examination. 
5 


CHAPTER  II. 
MENTAL  EXAMINATION  METHODS. 

In  no  department  of  medicine  is  a  complete  examination  of  the 
patient  more  important  than  in  the  department  of  psychiatry.  This 
examination  must  not  only  include  the  symptoms  that  the  patient 
may  present  when  seen,  but  must  also  include  the  most  detailed 
obtainable  anamnesis.  It  must  be  borne  in  mind  that  a  psychosis  is  a 
condition  of  an  individual  who  was  previously  well  and  that  above 
all  it  is  not  a  something  that  comes  from  without,  attacks  and  seizes 
on  the  patient  like,  for  example,  a  pathogenic  microorganism,  but  is 
rather  to  be  considered  as  a  type  of  reaction  of  the  individual  to  certain 
inimical  conditions.  In  order,  therefore,  to  understand  a  particular 
case  it  is  of  the  highest  importance  to  have,  as  fully  as  possible,  a 
conception  of  the  individual  before  he  became  afflicted,  so  that  the 
symptoms  which  are  the  expressions  of  this  reaction  may  be  under- 
stood. 

The  scheme  of  examination  which  follows  is  directed  primarily  to 
elucidating  the  mental  state.  It  is  taken  for  granted  that  the  student 
is  familiar  with  the  various  methods  of  physical  examination.  The 
omission  of  specific  directions  as  to  the  physical  examination  is  not, 
however,  to  be  taken  as  an  indication  that  it  is  considered  unimportant. 
On  the  contrary  a  physical  examination  in  minute  detail  is  of  the  utmost 
importance  and  unless  it  is  made  the  risk  is  bound  to  be  run  that  the 
key  to  the  whole  situation  will  be  overlooked. 

Mental  disorders  at  best  are  obscure  phenomena  and  no  pains 
should  be  spared  to  illuminate  them  from  every  quarter.  It  is  not, 
of  course,  expected  that  every  possible  physical  test  will  be  applied 
to  each  case.  For  example,  it  would  be  quite  foolish  to  stain  for  the 
malarial  parasite  unless  there  was  some  clinical  evidence  of  malarial 
infection.  The  usual  examination  of  heart,  lungs  and  urine  should, 
however,  be  made  in  each  instance.  Similarly  with  the  neurological 
examination.  Trousseau's  or  Chvostek's  signs  would  hardly  be  thought 
of  unless  tetany  were  suspected,  while  in  every  case  the  reaction  of  the 
pupils  to  light  and  accommodation  and  the  patellar  tendon  reflex 
should  be  recorded  and  in  patients  suspected  of  organic  brain  disease 
or  paresis  the  Babinski  reflex  and  Biernacki's  sign  should  be  examined 
for. 

The  principal  value  that  a  scheme  of  examination  may  have,  how- 
ever, is  in  formulating  tests  that  call  for  an  actual  record  of  the  patient's 
reaction  and  not  the  conclusions  of  the  examiner.  Hospital  records 
are  filled  with  such  remarks  as  "the  patient  shows  lack  of  judgment" 


HISTORY  OF  PATIENT  67 

or  is  "disoriented"  or  has  ''failure  of  memory."  All  of  these  are 
conclusions  and  by  no  means  records  of  facts.  Such  histories  are 
useless  to  any  one  except  perhaps  the  persons  who  wrote  them.  The 
reader  of  a  history  is  entitled  to  a  statement  of  the  facts  on  which 
the  conclusion  is  based  and  then  he  is  at  liberty  to  form  his  own  con- 
clusion from  the  identical  premises.  How  much  better  and  more 
accurate  than  the  statement  "defective  memory"  would  be  this  test: 
The  patient  in  the  course  of  the  examination  is  given  the  address  375 
Oxford  St.  After  five  minutes  he  is  asked  to  recall  it.  He  gives 
the  number  176,  but  cannot  give  the  name  of  the  street  at  all.  Here 
is  a  definite  fact.  A  multiplicity  of  such  facts  gives  one  a  basis  for 
conclusions  about  the  patient.  Of  such  statements  should  the  record 
of  an  examination  be  composed. 

The  examination  will  be  considered  under  the  following  heads  :^ 
I.  Family  History. 
II.  History  of  Patient. 
HI.  Present  Illness. 
IV.  General  Observations. 
V.  Physical  Examination. 
VI.  Neurological  Examination. 
VII.  Mental  Examination. 

I.  Family  History. — Informant  (name,  relationship  to  patient, 
address). 

Grandparents;  parents  (uncles  and  aunts);  Siblings. 

Children  (with  abortions  and  miscarriages). 

Note. — ^In  securing  the  family  history  it  must  be  remembered  that 
it  is  equally  important  to  get  a  record  of  all  the  normal  members  of 
the  family  as  well  as  the  abnormal  and  not  stop  with  securing  the  latter, 
as  is  often  done.  The  patient's  relation  to  hereditary  tendencies  can 
only  be  determined  by  securing  the  fullest  information  about  his 
ancestors. 

II.  History  of  Patient. — Full  name:  address;  occupation. 
Birth;  childhood  diseases;  when  learned  to  walk  and  talk. 

Diseases  (especially  convulsions,  delirium,  head-injury,  gonorrhea, 
syphilis,  rheumatism,  neuritis). 

Habits  (alcohol,  drugs,  and  sexual). 

Marriage:  Menstruation;  gynecological. 

Previous  attacks  (special  attention  to  so-called  hysterical,  to  nervous 
breakdown,  and  to  melancholic  periods). 

Crimes  and  misdemeanors. 

Mental  make-up. 

Note. — The  history  of  the  patient  is  especially  important  for 
getting  a  comprehensive  idea  of  the  sort  of  person  the  patient  was 
before  becoming  ill.  A  given  mental  disorder  cannot  be  fully  under- 
stood without  understanding,  not  only  the  circumstances  that  gave 

'  Many  of  these  details  have  been  covered  in  the  neurological  questionnaire,  and  hence 
are  here  briefly  recapitulated  only. 


b8  MENTAL  EXAMINATION  METHODS 

rise  to  it,  but  the  other  and  more  important  factor,  the  make-up  of 
the  individual  in  whom  the  disorder  occurs. 

III.  Present  Illness. — Onset;  cause;  physical;  mental  and  moral 
changes;  emotional  condition;  hallucinations  and  delusions;  judgment; 
memory;  suicide  and,  homicide;  insight. 

Note. — Under  this  head  an  inquiry  is  made  into  all  the  circumstances 
surrounding  and  conditioning  the  onset  of  the  psychosis  and  the 
patient's  attitude  toward  them  and  his  insight.  He  should  be  asked 
frankly  whether  he  believes  himself  insane;  if  not,  how  is  it  that  he 
has  been  sent  to  a  hospital  for  the  insane;  what  he  may  have  done  or 
said  to  lead  others  to  think  him  insane;  what  is  his  explanation  of  the 
whole  situation  and  how  it  all  came  about. 

IV.  General  Observations. — Facial  expressioyi;  appearance  and 
demeanor;  movements;  speech;  mental. 

Note  : — The  general  observation  of  the  patient  is,  of  course,  always 
important:  whether  he  appears  silly,  resentful,  indifferent;  whether 
he  has  mannerisms,  etc.  It  is  particularly  important,  however,  in 
stuporous  and  delirious  patients  who  either  will  not  speak  or  are  not 
responsive  and,  therefore,  not  accessible.  These  patients  should  be  ob- 
served particularly  as  to  their  general  attitude  of  body  and  limbs,  the 
expression  of  the  face,  the  reflexes,  and  the  reactions — volitional,  emo- 
tional, and  organic  (hunger,  sexual,  responding  to  calls  of  nature,  etc.). 

V.  Physical  Examination. — Form;  nutrition;  iveight;  height;  skin; 
hones  and  joints. 

Decubitus;  scars  (especiall}^  penis  and  mouth) . 

Respiratory  system,. 

Circulatory  system;  heart-position,  size  and  sounds;   blood-pressure. 

Genito-urinary  system. 

Gastro-intestinal  tract;  stomach  content  (if  indicated);  glands;  abdomen. 

Sputum  (if  indicated) . 

Blood  composition  (if  indicated). 

Cerebrospinal  fluid  (if  indicated). 

Urine  (always). 

Note. — It  is  hardly  necessary  to  insist  upon  the  necessity  for  a 
thorough  phj'^sical  examination  in  every  case.  It  is  especiall}'^  impor- 
tant in  the  deliria  in  which  the  mental  disorder  may  be  the  expression 
of  an  obscure  physical  condition. 

VI.  Neurological  Examination. — (See  Chapter  I.)  Atrophy;  hyper- 
trophy. 

Movements,  voluntary:  activity;  rapidity,  accuracy,  force  (especially 
paresis) ;  limitations. 

Movements,  involuntary:  rigidity;  tremor  (at  rest,  intention); 
spasms;  convulsions. 

Reflexes:  KK;  TA;^  contralat.  add.;  plantar;  cremasteric  (inguinal); 
bladder;  anal;  epigastric;  triceps;  ulnar;  radial;  jaw;  clonus  (ankle, 
patellar  and  wrist). 

'  KK  =  knee-kick ;  TA  =  tendo-A chillis. 


MENTAL  EXAMINATION  '  69 

Nerve-trunk  sensitiveness;  tender  areas  (especially  vertebrae,  breast, 
ovarian). 

Coordination:  FN;  FF;  FT;  KH;^  station  (eyes  open  and  closed, 
one  and  both  feet) ;  gait. 

Sensations:  touch;  pain;  organic;  parasthesia;  hypesthesia;  hyper- 
esthesia; anesthesia;  analgesia;  sense  of  position;  feeling  of  reality. 

Cranial  nerves: 

1.  Smell:  solutions  and  subjective. 

2.  Hemiopia:  fundus;  hallucinations. 

3.  4,  6.  Eye  movements  (all  directions) :  squint;  diplopia;  ptosis; 

nystagmus  (horizontal,  vertical,  rotary).     Pupils:  size;  out- 
line; direct  light;  consensual  light;   accommodation;  sym- 
pathetic. 
5.  Corneal    reflex:  chewing  movements;   taste;    solutions   and 
subjective. 

7.  Facial  symmetry  (whistling);  tremors;  test-phrases. 

8.  Hearing:  objective  and  subjective  vertigo. 

9.  10,  11.  Swallowing:  pharyngeal  reflex. 
12.  Protrusion  of  tongue. 

Note. — In  a  condition  which  admittedly  involves  the  central 
nervous  organs,  particularly  the  brain,  the  neurological  examination 
becomes  of  the  greatest  importance.  Especial  importance  should  be 
paid  to  the  cranial  nerve  distributions  and  to  the  presence  of  paralyses 
or  anesthesias,  which  might  have  localizing  significance. 

VII.  Mental  Examination. — Orientation:  time;  place;  persons. 

General  memory:  family;  school;  occupation;  marriage;  children; 
diseases. 

Emotional  status:  insight;  sleep;  dreams. 

Hallucinations:  auditory;  visual;  other  senses. 

Speech:  voluntary;  writing  (name,  date,  the  United  States  of 
America,  the  Commonwealth  of  Massachusetts);  auditory;  visual;  test- 
phrases.     (Statistical,  perturbation,  Third  Riding  Artillerv  Brigade.) 

Stories  ("Cowboy,"  "Gilded  Boy,"  "Polar  Bear,"^  "Shark," 
"Good  Girl"). 

Special  memory:  Civil  War;  names  of  two  generals;  three  European 
countries;  capital  of  native  State;  President;  45319628;  35984271; 
487631;  955217;  7368;  487;  352;  375  Oxford  Street  (after  3  or  5 
minutes). 

Masselon  (hunter,  dog,  gun,  forest,  rabbit;  man,  wood,  coal,  stove, 
dinner ;  needle,  thread,  button,  vest ;  pipe,  match,  smoke ;  pen,  ink,  letter) . 

Ziehen  (horse  and  ox;  dwarf  and  child;  lie  and  mistake;  water  and 
ice).  7x6;  56-18;  23-14;  81-9;  x-5  =  17;  x-8=13;  have  50c; 
buy  cherries  12c,  butter  7c,  bread  10c;  how  much  change? 

Forward  and  backward  associatiojis  (month;  days  of  week;  752186, 
25729,  6418,  265,  497). 

1  FN  =  finger-nose;  FF  =  finger-finger;  FT  =  finger-thumb;  KH  =  knee-heel. 


70  MENTAL  EXAMINATION  METHODS 

General  information:  cost  of  postage;  color  of  stamps;  holidays  and 
meaning  (Christmas,  Easter,  Fourth  of  July). 

Finckh  ("The  early  bird  catches  the  worm;"  "Lies  have  short  legs;" 
"Set  a  thief  to  catch  a  thief;"  "Burn  a  candle  at  both  ends"). 

Ethical  questions: 

Drawing  diagram  (after  five  seconds'  exposure). 

Note. — Here  especial  caution  is  needed  to  avoid  recording  conclu- 
sions. For  example:  Under  orientation  the  patient's  actual  answers 
to  such  questions  as.  When  were  you  born?  How  old  are  you?  What 
day  is  this?  etc.,  should  be  put  down. 

The  stories  which  are  named  are  as  follows : 

"Cowboy  Story." — A  cowboy  from  Arizona  went  to  San  Francisco 
with  his  dog,  which  he  left  at  a  dealer's  while  he  purchased  a  new 
suit  of  clothes.  Dressed  finely,  he  went  to  the  dog,  whistled  to  him, 
called  him  by  name  and  patted  him.  But  the  dog  would  have  nothing 
to  do  with  him  in  his  new  hat  and  coat  but  gave  a  mournful  howl. 
Coaxing  was  of  no  effect,  so  the  cowboy  went  away  and  donned  his  old 
garments,  whereupon  the  dog  immediately  showed  his  wild  joy  on 
seeing  his  master  as  he  thought  he  ought  to  be. 

"Gilded  Boy  Story." — It  is  related  that  at  the  coronation  of  one 
of  the  popes,  about  three  hundred  years  ago,  a  little  boy  was  chosen  to 
act  the  part  of  an  angel;  and  in  order  that  his  appearance  might  be 
as  gorgeous  as  possible  he  was  covered  from  head  to  foot  with  a  coating 
of  gold  foil.  He  was  soon  taken  sick,  and  although  every  known 
means  was  employed  for  his  recovery,  except  the  removal  of  his  fatal 
golden  covering,  he  died  within  a  few  hours. 

"  Polar  Bear  Story." — A  female  polar  bear  with  two  cubs  was  pursued 
by  sailors  over,  an  ice  field.  She  urged  her  cubs  forward  by  running 
before  them,  and,  as  it  were,  begging  them  to  come  on.  At  last  in 
dread  of  their  capture  she  pushed,  then  carried  and  pitched  each 
before  her,  until  they  actually  escaped.  The  polar  bear  is  a  wonderful 
swimmer  and  diver.  In  the  capture  of  seals  lying  on  the  ice,  it  dives 
some  distance  off  and  swimming  underneath  the  water,  suddenly 
comes  up  close  to  the  seals,  shutting  off  their  retreat  to  the  sea. 

"Shark  Story." — The  son  of  a  governor  of  Indiana  was  first  officer 
on  an  Oriental  steamer.  When  in  the  Indian  Ocean  the  boat  was 
overtaken  by  a  typhoon  and  was  violently  tossed  about.  The  officer 
was  suddenly  thrown  overboard.  A  life  preserver  was  thrown  to 
him,  but,  on  account  of  the  heavy  sea,  difficulty  was  encountered  in 
launching  the  boat.  The  crew,  however,  rushed  to  the  side  of  the 
vessel  to  keep  him  in  sight,  but  before  their  shuddering  eyes  the  unlucky 
young  man  was  grasped  by  one  of  the  sharks  encircling  the  steamer 
and  was  drawn  under  the  water,  leaving  only  a  dark  streak  of  blood. 
(Adapted  from  Ziehen.) 

"Good  Girl  Story." — Once  upon  a  time  there  was  a  girl,  whose 
father  and  mother  were  dead,  and  who  was  so  poor  that  finally  she 
had  nothing  but  the  clothes  on  her  back  and  a  little  piece  of  bread  in 


MENTAL  EXAMINATION  71 

her  hand.  She  was  deserted  by  everybody,  but  since  she  was  good  and 
honest  she  went  into  the  world  with  confidence  in  God.  As  she  went 
along  she  was  met  by  a  poor  old  man  who  said,  "Give  me  something 
to  eat,  I  am  hungry."  The  girl  gave  him  the  piece  of  bread  and  went 
on  farther.  Soon  afterward  she  encountered  a  little  girl  freezing 
and  almost  naked,  who  begged  for  her  clothes.  The  good  girl  gave  the 
poor  child  the  warmest  of  her  garments.  Night  came  on,  the  good  girl 
was  tired,  cold,  and  hungry.  She  traveled  into  the  woods,  and,  wander- 
ing off  the  road,  she  knelt  and  prayed  to  God.  As  she  knelt  she  saw 
the  stars  falling  all  about  her,  and  when  she  looked  she  found  they  were 
many  bright  gold  dollars.     (Adapted  from  Ziehen.) 

These  stories  which  are  used  have  been  selected  with  great  care. 
They  are  especially  valuable.  It  is  remarkable  the  amount  of  informa- 
tion that  one  can  obtain  from  getting  a  patient  to  repeat  one  or  two. 
Defects  of  memory  and  attention  show  immediately,  while  the  manic 
tendency  to  elaborate  is  characteristic.  They  should  never  be  omitted. 
The  cowboy  story  is  usually  the  easiest,  while  the  good  girl  story  is 
hard,  because  of  the  great  amount  of  detail.  The  emotional  feature 
of  the  "streak  of  blood"  in  the  shark  story  is  particularly  impressive 
and  may  be  about  the  only  feature  of  the  story  reproduced. 

In  the  special  memory  test,  of  course,  different  people  will  have  to  be 
treated  differently.  A  Polish  immigrant  just  landed  would  hardly 
know  about  the  Civil  War.  The  important  thing,  however,  is  to 
record  actual  question  and  answer. 

In  the  Masselon  tests  the  patient  is  asked  to  incorporate  such 
words  as  pen,  ink,  letter,  into  a  sentence. 

In  the  Ziehen  test  the  patient  is  asked  to  tell  the  difference  between 
horse  and  ox,  dwarf  and  child,  etc. 

The  problem  of  calculating  the  change  left  from  50c  after  making 
certain  purchases  is  an  excellent  example  of  the  usefulness  of  standard 
questions.  Every  one  on  the  hospital  staff  knows  that  the  answer 
is  21c;  and  although  this  is  a  little  thing,  when  multiplied  many  times 
it  makes  a  great  deal  of  difference  in  the  ease  with  which  one  can  go 
over  a  history  or  appreciate  it  when  read. 

The  forward  and  backward  associations  are  valuable  as  roughly 
quantitative.  The  average  person  should  be  able  to  give  six  numbers 
forward  and  five  numbers  backward.  This  test  will  disclose  just  how 
many  the  patient  can  give  and  is  one  of  the  valuable  tests  for  repeating 
from  time  to  time  during  the  course  of  the  psychosis.  It  is  also  very 
valuable  in  detecting  the  malingerer.  A  definite  intention  to  blunder 
is  usually  readily  distinguishable  from  a  natural  blunder. 

In  the  Finckh  test  the  patient  is  asked  the  meaning  of  the  several 
sayings  such  as  "The  early  bird  catches  the  worm." 

Such  ethical  questions  can  be  asked  as  What  would  you  do  if  you 
saw  a  man  drop  a  SIO  bill? 

In  addition  to  the  tests  given  in  the  table  frequent  use  is  made  of 
the  Ebbinghaus  test  which  consists  of  having  the  patient  complete  a 


72  MENTAL  EXAMINATION  METHODS 

sentence  in  which  certain  words  have  been  left  out,  such  as:  I  got  up 
in  the  .  .  .  ,  and  after  washing  my  .  .  .  went  to  .  .  .  Or 
better  often  is  Ziehen's  modification  of  this  test.  .  The  patient  is 
asked  to  complete  such  a  sentence  as  this:  If  it  rains  .  .  .  because 
.  .  .  in  spite  of  .  .  .  The  Bourdon  test  is  very  valuable  as  a 
measure  of  attention.  It  consists  of  getting  the  patient  to  strike  out 
certain  recurring  letters  or  numbers  in  a  standard  page  and  timing  the 
result.  A  similar  test  is  the  tapping  test — timing  the  number  of  taps 
that  can  be  made  in  a  given  time,  say  thirty  seconds. 

Of  course  the  cases  will  be  numerous  in  which  it  will  be  found  desir- 
able or  necessary  to  pursue  the  examination  further  in  some  direction. 
No  scheme  can  cover  all  possibilities  and  would  be  useless  if  it  did, 
because  impossible  to  carry  out.  Much  must  of  necessity  be  left  to 
the  judgment  of  the  examiner.  By  following  this  plan,  however,  it  is 
believed  that  the  general  and  important  features  necessary  for  a  case 
record  will  be  covered  in  the  large  majority  of  cases. 

It  is  useful,  after  completing  the  examination,  to  accent  the  sig- 
nificant features  in  a  short  summary,  which  might  include  a  provisional 
diagnosis  if  the  facts  warranted. 

The  tests  described  above  are  for  the  most  part  intelligence  tests. 
Even  such  questions  as  might  be  propounded  under  the  head  of  "  ethical 
questions"  may  very  easily  have  only  the  value  of  intelligence  tests 
because  the  patient  will  quite  likely  answer  in  accordance  with  the 
conventional  ideas  with  which  he  is  perfectly  familiar  rather  than 
answer  in  accordance  with  the  way  in  which  he  feels.  While  the  intelli- 
gence tests  are  important  and  while  by  taking  them  in  a  routine  manner 
one  can  get  a  good  deal  of  information  from  the  patient,  often  informa- 
tion of  matters  that  lie  deeper  than  mere  questions  of  intelligence,  still 
they  are  by  no  means  all  sufficient.  The  intellect  is  after  all  only 
superficial  as  a  guide  to  conduct.  The  deeper  motives  that  move  men 
to  action  come  from  the  realm  of  feeling,  and  if  the  symptoms  are  to 
be  explained  or  understood  the  emotional  springs  of  conduct  must 
be  fathomed. 

PSYCHOANALYSIS. 

Psychoanalysis  is  the  method  by  which  the  human  mind  is,  so  to 
speak,  dissected,  and  by  means  of  which  the  hidden  motives  of  conduct 
are  sought.  If  anything  like  a  complete  understanding  of  patients 
is  to  be  had  the  methods  of  psychoanalysis  must  be  used.  This  is 
hardly  the  place  to  discuss  these  methods  at  length.  It  would  require 
more  space  than  a  text-book  of  this  character  could  properly  give  it. 
The  student  is  referred  to  special  works.  Here  only  will  be  given  the 
briefest  suggestions.^ 

The  Complex. — The  mind  cannot  be  conceived  of  as  consisting  of 
or  containing  ideas  which  are  deposited  here  and  there,  helter  skelter, 

1  Hitschmann,  Freud's  Theories  of  the  Neuroses,  New  York;  Jung,  Theory  of  Psy- 
choanalysis, New  York;  Psychoanalytic  Review,  New  York. 


PSYCHOANALYSIS  73 

without  order,  as  the  scraps  of  paper  that  are  thrown  carelessly 
into  a  waste  basket.  Quite  the  contrary.  Ideas  are  grouped  about 
central  experiences,  constellated  one  may  say,  built  into  coherent  and 
harmonious  structures  not  unlike  the  way  in  which  bricks  and  stones 
are  brought  together  to  form  buildings  and  these  buildings  are  again 
grouped  to  form  the  larger  whole — the  city.  The  significant  fact  in 
this  connection  is  that  the  cement  that  holds  the  bricks  and  stones 
together,  the  binding  substance,  h  feeling. 

This  orderly  arrangement  of  ideas  upon  a  background  of  feeling 
which  serves  to  unite  them  is  what  gives  character,  individuality  to 
the  personality.  The  creating  of  the  proper  feeling-tone  about  things 
and  events  is  one  of  the  main  functions  of  education. 

Now  it  so  happens  that  in  certain  types  of  individuals  a  constellation 
of  ideas,  grouped  about  a  central  event  that  conditions  a  highly  painful 
emotional  state,  is  crowded  out  of  clear  consciousness — repressed — into 
the  unconscious  and  so  tends  to  lead  an  existence  which  is  relatively 
independent  and  in  so  doing  gives  origin  to  various  symptoms.  Such 
a  constellation  is  a  "complex." 

The  complex,  crowded  out  of  relation  with  the  personal  conscious- 
ness, seeks  for  expression  and  because  it  is  not  synthetized  with  the 
rest  of  consciousness,  because  the  individual  is  not  aware  of  its  exist- 
ence, its  expression  cannot  be  controlled  and  guided  into  the  usual 
channels  and  so  creates  symptoms. 

The  extreme  difficulty  in  locating  and  uncovering  the  complex  is 
due  to  the  symbolic  forms  in  which  it  usually  manifests  itself.  The 
painful  memories  of  disagreeable  experiences,  unethical,  unconven- 
tional, and  otherwise  impossible  and  hateful  wishes  while  crowded  out 
of  mind  by  what  Freud  has  so  aptly  termed  the  "censor  of  conscious- 
ness" nevertheless  struggle  to  find  expression.  The  complex  cries  for 
recognition,  the  censor  will  have  none  of  it — the  fight  is  on,  the  conflict 
wages,  until  finally  a  sort  of  compromise  is  reached  by  permitting  the 
complex  to  come  into  clear  consciousness  but  only  on  pain  of  not  dis- 
closing its  true  self,  under  the  cloak  of  a  complete  disguise. 

For  example  Freud's  case  of  Elizabeth.  She  was  engaged  in  nursing 
her  sick  father  who  afterward  died.  One  evening,  spent  away  from 
home  at  the  solicitation  of  the  family,  she  met  a  young  man  of  whom 
she  was  very  fond  and  he  accompanied  her  back  home.  On  the 
walk  home  she  quite  gave  herself  up  to  the  happiness  of  the  occasion 
and  walked  along  oblivious  of  her  duties.  On  reaching  home  she 
found  her  father  much  worse  and  bitterly  reproached  herself  for  for- 
getting him  in  her  own  pleasure.  She  immediately  repressed  this 
disagreeable  thought  from  her  consciousness.  Now  she  had,  each 
morning,  to  change  the  dressings  on  her  father's  swollen  leg.  To  do  this 
she  took  his  leg  upon  her  right  thigh.  The  suppressed  complex  seized 
upon  the  feeling  of  weight  and  pain  of  her  father's  leg  upon  her  thigh 
as  a  hand}'  and  efficient  means  of  expression  and  so  the  repressed  erotic 
wish  comes  into  consciousness  under  the  disguise  of  a  painful  area  of  the 


74  MENTAL  EXAMINATION  METHODS 

right  thigh  corresponding  in  extent  and  location  to  the  place  upon 
which  she  rested  her  father's  leg. 

This  is  the  sort  of  mechanism  that  accounts  for  many  unusual  and 
strange  experiences  that  otherwise  appear  to  be  without  reason. 
Unexplained  forgetting,  slips  of  the  tongue,  certain  mental  attitudes, 
moods,  and  even  the  dominant  traits  of  character  are  due  to  the 
activity  of  submerged  complexes  while  the  phenomena  of  dreams  are 
explained  in  the  same  way. 

The  unconscious  methods  are  very  logical.  As  already  described 
the  complex  often  expresses  itself  symbohcally  (symbolism),  often  by 
the  transfer  of  an  emotion  from  a  painful  event  to  a  less  painful  or 
indifferent  event  {displacement),  often,  as  in  hysteria,  by  the  conversion 
of  the  conflict  into  a  physical  symptom  (conversion). 

Dreams. — The  analysis  of  dreams  is  for  the  purpose  of  determining 
the  presence  and  nature  of  complexes  which  are  exercising  a  controlling 
effect  upon  the  patient's  conduct  and  feelings.  The  dream  appears  as  a 
quite  senseless  experience  to  the  patient  and  upon  the  face  of  it  it 
would  appear  also  to  be  senseless.  A  very  little  effort,  however,  will 
show  that  there  is  a  certain  rough  meaning  to  the  dream.  For  example, 
the  scenes  of  the  dream  will  be  representations,  usually  more  or  less 
fragmentary,  of  things  which  have  happened  in  the  life  of  the  individual 
during  the  previous  twenty-four  hours  and  may  easily  be  associated 
in  his  mind  with  events  of  some  moment  to  him.  As  soon  as  this  is 
pointed  out  the  patient  will  acknowledge  it,  if  he  does  not  know  it 
first  himself,  and  will  think  that  the  dream  has  been  explained.  This, 
however,  is  only  the  manifest  content  of  the  dream.  Behind  this 
manifest  content  there  lies  a  deeper,  a  more  profound,  a  more  important 
meaning  or  series  of  meanings.  The  superficial  experiences  only  serve 
to  hide  the  latent  content,  which  contains  the  real  material  of  importance 
in  the  dream. 

The  latent  content  will  almost  invariably  show  that  the  dream  in 
its  deeper  meanings  is  dealing  with  repressed  material,  with  material 
that  the  patient  is  not  willing  to  acknowledge  to  himself.  The  whole 
object  of  the  dream  is  primarily  to  conserve  sleep,  and  to  that  end 
it  does  not  permit  the  real  dream  thoughts  to  become  known,  and  so 
builds  up  a  structure  that  upon  its  surface  appears  utterly  without 
meaning  and  nonsensical,  and  therefore  is  hardly  deserving  of  attention. 
Therefore  the  dreamer  usually  passes  the  dream  by  without  any 
particular  notice,  and  in  the  course  of  the  day  it  is  generally  forgotten. 

The  mechanisms  by  which  this  disguise  is  effected  have  already  been 
spoken  of  in  discussing  the  complex.  The  mechanisms  are  those  of 
distortion.  These  mechanisms  of  distortion  are  first  the  mechanism  of 
disphcement,  which  effectuates  the  transfer  of  the  principal  affect  from 
the  situation  where  it  belongs  to  some  other  situation.  The  feeling 
of  anxiety,  or  fear,  or  disgust,  if  it  were  retained  attached  to  that 
portion  of  the  dream  to  which  it  is  normally  attached,  would  disclose 
to  the  patient  the  real  thing  about  which  he  was  anxious,  fearful,  or 


PSYCHOANALYSIS  75 

disgusted,  but  if  displaced  upon  some  indifferent  part  of  the  dream 
serves  the  purpose  of  distortion.  Overdetermination  is  a  mechanism 
which  secures  the  appearance  in  the  dream  of  a  particular  figure,  for 
example  because  of  its  likeness  not  to  any  one  experience  in  the  patient's 
life,  but  to  man3^  All  of  these  experiences,  as  it  were,  focus  in  one 
direction  and  produce  the  result;  for  example  the  face  of  a  dreamed-of 
person  may  appear  to  be  entirely  unknown  to  the  dreamer,  but  when 
it  is,  so  to  speak,  dissected  it  is  discovered  that  each  portion  of  the 
face,  the  nose,  the  lips,  the  eyes,  the  hair,  each  of  them,  have  well 
defined  associations  in  the  mind  of  the  dreamer  and  that  they  have  all 
condensed  into  this  composite  to  serve  the  purpose  of  the  dream 
formation.  Symbolism  is  another  mechanism  which  aids  distortion 
so  that  instead  of  things  being  dreamed  of  as  they  are  in  reality,  they 
are  dreamed  of  as  being  represented  symbolically,  and  so  the  dream 
becomes  increasingly  difficult  to  read,  and  to  the  dreamer  quite  unin- 
telligible. Further,  the  mechanism  of  secondary  elaboration  is  the 
mechanism  by  which  the  dreamer,  after  he  is  awake,  goes  on,  as  it  were, 
with  the  dream  material  and  explains  to  himself  by  a  process  of  ration- 
alization what  certain  features  of  the  dream  mean,  and  still  further 
distorts  it  and  places  it  beyond  the  pale  of  his  power  to  recognize  it. 

Here  at  work  in  the  dream  is  seen  one  of  the  most  exquisite  of 
defense  mechanisms  of  the  human  being.  One  is  accustomed  to 
think  of  the  human  animal  biologically  as  defending  itself  from  all 
sorts  of  attacks  upon  its  integrity.  The  various  infections  by  micro- 
organisms are  met  by  vital  processes  of  defense  which  are  frequently 
fully  successful,  and  the  whole  theory  of  immunity  is  the  theory  of 
a  defense  mechanism  of  the  human  animal.  The  dream  is  just  such  a 
mechanism,  and  if  what  is  going  on  in  the  mind  of  the  patient  would 
be  known,  what  the  patient  is  defending  himself  from,  what  are  the 
disintegrating  factors  at  work  at  the  psychological  level,  the  easiest 
access  to  the  knowledge  of  these  factors  can  be  found  if  the  meanings 
of  the  dream  can  be  learned.  Dream  analysis  is  a  most  important 
tool  for  the  unravelling  and  treatment  of  all  of  the  neuroses  and 
psychoneuroses  and  for  the  understanding  of  the  psychoses. 

Technique. — The  technique  of  psychoanalysis  is  an  art  more  success- 
ful in  some  hands  than  in  others.  However,  the  general  methods  of 
procedure  may  be  briefly  outlined. 

In  the  first  instance  the  physician  must  be  fully  imbued  with  a 
profound  belief  that  mental  symptoms  have,  each  and  every  one  of 
them,  a  meaning  and  a  meaning  which  can  be  brought  to  light  and  will 
show  them  to  be  logical  and  understandable  in  each  instance.  He 
must  then  have  patience  to  listen  to  the  story  of  his  patient,  and  not 
only  listen  to  it,  but  listen  to  it  attentively  for  the  purpose  of  trying 
to  find  the  meaning  in  it,  for  the  purpose  of  trying  to  find  out  where 
the  vital  points  are  which  can  be  attacked  to  best  advantage. 

It  is  true  that  the  dream  analysis  is  the  main  avenue  to  the  under- 
standing of  the  unconscious  motives  of  action,  but  all  sorts  of  hints 


76  MENTAL  EXAMINATION  METHODS 

may  come  from  other  sources.  For  example  one  of  the  authors  was 
recently  listening  to  the  story  of  a  patient.  In  the  course  of  that  story 
the  patient  mis-spoke  and  said  quinine  when  he  intended  to  say  calomel. 
Realizing  that  a  "  slip  of  the  tongue"  of  this  sort  must  have  its  meaning 
and  is  not  an  accidental  occurrence,  because  nothing  mental  is  acci- 
dental, the  slip  of  the  tongue  was  analyzed  and  led  directly  back  to 
one  of  the  most  important  emotional  events  in  the  life  of  the  patient, 
an  event  which  threw  a  flood  of  light  upon  his  psychoneurosis. 

Nothing  is  too  trivial  to  be  worthy  of  analysis,,  nothing  but  may 
throw  light  upon  the  situation.  All  the  little  slips  of  the  tongue, 
forgotten  incidents,  points  at  which  two  recitals  of  an  occurrence  do 
not  agree,  even  witticisms  are  necessary  to  trace  out  besides  the 
analysis  of  the  dream  life,  and  offer  an  abundance  of  material  in  the 
course  of  the  analysis. 

The  method  of  procedure  is  the  method  of  free  association,  whether 
it  be  the  analysis  of  some  component  of  a  dream  or  of  a  slip  of  the 
tongue,  or  what  not,  the  method  of  free  association  is  the  one  employed. 
The  patient  should  be  alone  with  the  physician.  It  is  practically 
impossible  to  conduct  an  analysis,  at  least  beyond  the  surface,  in  any 
other  way.  Under  circumstances  of  quiet  and  freedom  from  interrup- 
tion, as  far  as  possible,  the  different  points  which  are  to  be  analyzed 
are  taken  up.  The  patient  is  instructed  to  take  a  certain  element 
of  the  dream  which  he  has  just  recounted,  for  example,  and  hold  it 
in  his  mind,  and  then  tell  freely  all  of  the  ideas  that  come  to  him.. 
He  is  told  to  tell  all  of  the  ideas  without  any  effort  on  his  part  of 
selection,  no  matter  whether  the  ideas  appear  to  him  to  have  any 
relationship  with  the  portion  of  the  dream  that  he  has  been  told  to 
keep  in  mind  or  not,  and  no  matter  whether  they  appear  ridiculous 
or  have  other  qualities  that  incline  him  to  lay  them  aside.  He  must 
tell  them  all  just  as  a  man  might  sit  at  the  window  of  a  railroad 
train  and  jot  down,  as  far  as  possible,  everything  that  he  sees  pass  the 
windows  as  the  train  speeds  on. 

The  theory  of  this  procedure  is  that  if  the  patient  does  not  direct 
the  thought  in  any  way  every  idea  that  comes  must  of  necessity  have 
some  relation  to  the  event  held  before  the  mind  about  which  enlight- 
enment is  sought.  This  is  the  method  of  unraveling  the  tangled  net- 
work of  the  mental  life  and  while  it  may  be  supplemented  by  word 
association  or  other  means,  still  it  would  seem  as  our  experience 
increases,  that  no  other  method  is  needed,  that  this  answers  all  the 
purposes.  It  takes  a  long  time,  as  a  rule,  however,  to  effect  an  analysis, 
— weeks,  months,  perhaps  longer.  It  must  be  remembered  that  what 
has  taken  years  to  form  cannot  be  unraveled  in  an  hour. 

It  will  probably  occur  to  many  to  wonder  how  it  is  that  one  can 
expect  to  find  memories  reaching  back  for  years  sufficiently  well 
preserved  to  be  helpful.  As  a  matter  of  fact  the  memories  of  all 
repressed  experiences  are  perfectly  clear  no  matter  how  old.  The 
explanation  for  this  is  that  being  repressed  they  are  dissociated  from 


PSYCHOANALYSIS  77 

the  everyday  events  of  life,  they  are  kept  in  their  original  form,  they 
have  not  been  subjected  to  the  attrition  and  amalgamation  with  the 
intricacies  of  associational  life.  They  do  not  fade  out  by  this  process 
of  absorption  as  do  the  memories  of  indifferent  events,  but  remain 
where  ever  after  they  may  be  brought  to  light  by  analysis  and  used  as 
helps  for  cure. 

It  will  be  seen  from  this  short  description  what  a  far-reaching 
method  this  is.  A  method  of  analysis  from  which  no  event  of  life, 
no  matter  how  apparently  trivial,  is  free.  A  method  that  in  its 
results  lays  bare  not  only  the  immediate  antecedents  and  causes  of  the 
symptoms,  but  the  whole  innermost  life  of  the  patient,  reaching  back 
even  to  the  period  of  early  childhood.  This  of  course  takes  time. 
A  case  of  any  complexity  and  difficulty  quite  generally  takes  several 
months,  of  at  least  three  seances  each  week,  to  reach  a  final  result. 


PART  I. 

PHYSICO-CHEMICAL  SYSTEMS. 


CHAPTER  III. 
VEGETATIVE  OR  VISCERAL  NEUROLOGY. 

THE  AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS— 
THE  INTERNAL  SECRETIONS. 

A  THOROUGHLY  consistent  plotting  of  this  enormous  chapter  in 
neurology  is  not  yet  possible.  AH  of  the  disorders  here  brought 
together  may  not  ultimately  be  found  best  grouped  here.  The  general 
assumption  followed,  however,  is  that  they  all  belong  to  disorders  of  a 
particular  group  of  organs,  partly  nervous,  partly  glandular,  but  which 
are  all  governed  by  a  homogeneous  series  of  structures,  the  so-called 
vegetative  nervous  system. 

Certain  of  these  organs  are  probably  closely  related  to  nervous 
structures,  hypophysis,  pituitary,  parathyroids,  suprarenals,  blood 
glands,  etc.,  and  have  been  variously  brought  together  as  the  chromaf- 
fine  system  or  the  endocrinous  or  blood  glands — while  others  are 
distinctly  non-nervous,  liver,  pancreas,  ovaries,  uterus,  lungs,  stomach, 
heart,  etc.,  but  their  functions  are  automatically  regulated  by  one  or 
other  of  the  two  portions  of  the  vegetative  system,  the  sympathetic 
proper,  and  the  parasympathetic  or  autonomic.  This  twofold  division 
of  the  vegetative  system  will  be  here  adopted.^ 

The  vegetative  nervous  system  consists  of  those  nervous  structures 
which  supply,  by  afferent  and  efferent  pathways,  impulses  to  the  special 
sense  organs,  smooth  muscle  fibers,  and  all  those  automatically  working 
organs  such  as  the  heart,  lungs,  intestines,  genital  apparatus,  blood- 
vessels, excretory  glands,  skin  and  organs  of  external  and  internal 
secretion,  such  as  the  Hver,  stomach,  pancreas,  intestinal  glands,  and 
the  thyroid,  thymus,  adrenals,  parathyroid,  hypophysis  and  epiphysis, 
etc.,  respectively.  All  of  these  structures  are  constantly  in  function, 
and  their  disturbances  are  manifold;  either  through  affect  activities  as 

1  See  Higier,  Vegetative  oder  Viscerale  Neurologie.  Ergebnisse  der  Neurologie  und 
Psychiatric,  vol.  2,  No.  1,  1912.  For  a  complete  discussion  of  this  generalization  see 
Eppinger  and  Hess,  Vagotonia.  Nervous  and  Mental  Disease  Monograph.  20.  New 
York,  1915. 


80 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


seen  in  many  neuroses  and  psychoneuroses  as  palpitation  of  the  heart, 
anorexia,  fainting,  crying,  diarrhea,  mydriasis,  etc.,  or  in  infections  or 
intoxications  as  reddening,  swelh'ng,  gooseflesh,  tachycardia,  dryness 
of  the  mouth,  stenocardia,  gastric  or  visceral  crises,  Argyll-Robertson 
pupil,  etc. 

The  vegetative  nervous  system  in  its  essence  is  the  primitive, 
archaic  rest  of  the  ganglionic  or  metameric  system  of  the  lower  verte- 
brates. Its  chief  switchboard  is  in  the  midbrain.  In  the  evolution 
of  higher  animals  its  development  has  been  left  behind,  as  it  were, 
by  the  relatively  more  important  (i.  e.,  for  purposes  of  civilization 
and  culture)  neopallium  or  cortex  with  its  rich  cortical-association 
system,  but  with  which  it  has  remained  in  close  relationship,  since 
these  structures  producing  consciousness  and  intelligence  have  grown 
out  of  the  phylogenetically  older  system.  Thus  it  comes  about  that 
the  ganglionic  system  which  in  man  serves  the  vegetative  functions  of 


Spinal  Ganglion 

Skin  Sensibility 

N.  Spinalis 

-'i-^ Sweat  Gland 

~\\\     i;i^-  \     —  Pilvmotor 

I  i 

:  !r§l,'l  '-^  V  Striped  Miiacle 

III  I' I  ^ 

/V//cf 


Fig.  25. — Diagram  of  the  central  connections  and  peripheral  distribution  of  the  vegeta- 
tive system, ;  the  motor,  — ,  and  the  sensory ,  fibers.     (Higier.) 


the  body  is  represented  in  the  primary  metameres,  the  spinal  cord, 
again  in  the  brain  stem,  central  gray  matter  and  midbrain,  lenticular 
nucleus  and  optic  thalamus,  and  finally  in  the  cortex. 

The  central  or  spinal  synapses  are  probably  located  in  Clark's  columns 
and  in  the  lateral  gray  of  the  spinal  cord  (Jacobsohn).  From  here  the 
centrifugal  fibers  pass  through  the  anterior  roots  to  and  from  the 
sympathetic  vertebral  ganglion  as  rami  communicans  albus  and  rami 
communicans  griseus  respectively  to  be  distributed  to  the  vessels, 
glands,  unstriped  muscles,  etc.,  after  the  sympathetic  break  in  the 
ganglion.  This  is  the  original  type  for  each  metamere,  the  symmetry 
of  which,  however,  has  been  much  distorted,  either  showing  as  an 
irregular  or  inconstant  localization  of  the  ganglia,  or  synapses,  incon- 
stancy of  the  communicating  branches,  irregular  distribution  of  the 
centers  in  the  cerebrospinal  axis  or  incongruity  of  the  embryonal 
metameres  with  the  spinal  and  cranial  segments  (Figs.  25-27). 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS        81 

The  chief  anatomical  results  may  be  recapitulated  as  follows:  In 
the  skull  the  distortion  is  greatest  as  many  metameres  are  anatomical 
conglomerates.  Man}'  of  the  ganglia  are  modified  intervertebral 
ganglia,  such  as  the  geniculate  and  the  Gasserian;  others  are  compound 
ganglia  due  to  the  amalgamation  of  a  spinal  with  a  sympathetic  ganglion 
as  the  jugular  and  vagus  ganglia;  other  pure  intervertebral  ganglia 
are  the  ciliary,  otic,  sphenopalatine,  submaxillary  and  sublingual 
which  supply  the  smooth  muscle  of  the  eye,  the  vessels,  the  tear, 
salivary  and  mucous  glands  respectively.  Some  of  the  chief  anatomical 
features  for  the  head  ganglia  may  be  seen  in  the  superb  charts 
from  Miiller.^  The  upper  ganglion,  ganglion  cervicale  supremum, 
obtains  its  precellular  fibers  from  the  last  cervical  (C8)  and  upper 
dorsal  (D  1-3)  segments  and  innervates  the  vessels,  hair  muscles  and 


~{^:^,   Nucleus   SYmpath 
>*i^^  lateralis     Supe.r 


X;.zrr 


S.  12- . 


Fig.  26. — Sympathetic  nuclei  at  the  seventh  dorsal  and  fourth  sacral  levels  of  the 
spinal  cord.    (Timme,  Jour.  Nerv.  and  Ment.  Dis.,  1914.) 

skin  glands  of  the  head,  the  dilator  pupillse  and  Miiller's  orbital  muscle. 
The  inferior  cervical  ganglion  with  its  closely  related  stellate  ganglion 
derives  its  pregangliar  fibers  from  the  D  1-5,  and  gives  rise  to  the 
accelerator  nerve  of  the  heart  and  probably  vasoconstrictor  fibers  of 
the  pulmonary  vessels. 

The  largest  ganglion  of  the  abdomen,  the  celiac,  has  its  chief  root  in 
the  celiac  plexus  in  the  major  and  minor  splanchnic  nerves,  the  former 
of  which  comes  from  D  4-9,  the  latter  from  D  10-12.  As  the  mesenteric 
nerves  they  innervate  the  stomach  glands,  liver,  pancreas,  spleen, 
kidneys,  adrenals,  and  intestine  as  far  as  the  ascending  colon.  The 
inferior  mesenteric  ganglion  receives  its  precellular  fibers  from  L  1-3 
and  sends  its  postganglionic  fibers  to  the  colon  and  as  the  hypogastric, 
in  part,  to  the  anus,  bladder,  sphincter  of  bladder  and  genitals. 


'  Higier,  loc.  cit. 


82 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


A  series  of  blood  glands,  chromaffine  cell  containing  structures 
(paraganglia)  have  of  late  been  regarded  as  closely  related  to  these 
clearly  recognized  ganglia  of  the  vegetative  system.  The  most 
important  of  these  are:  (a)  Paraganglion  caroticum,  (6)  paraganglion 
coccygeus,  (c)  paraganglion  aorticum,  and  (d)  paraganglion  supra- 
renalis  or  adrenals. 


7i;.T 


/ ,  Gut. 


S.^  ii^.v* 


Fig.  27. — Distribution  of  the  various  divisions  of  the  vegetative  system.  Sympathetic 
fibers  are  indicated  by  unbroken  lines;  parasympathetic  (autonomic)  by  dotted  lines. 
(Timme,  loc.  cit.) 


Sympathetic  and  Autonomic  Division. — Anatomically  as  well  as 
pharmacologically  it  appears  that  two  types  of  physiological  activity 
are  present  in  the  vegetative  nervous  system.  These  are  the  sympa- 
thetic and  the  autonomic.  All  those  non-voluntarily  influenced 
organs,  smooth  muscle  structures,  heart  muscle,  glands,  whose  nerve 
fibers  are  derived  from  the  spinal  cord  from  the  first  dorsal  above  to 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS        83 

the  fourth  lumbar  segment  below,  belong  in  the  sympathetic  system 
in  the  narrower  sense.  All  others  are  controlled  through  the  para- 
sympathetic or  autonomic.  The  uppermost  come  from  the  midbrain, 
enter  the  ciliary  ganglion,  and  are  distributed  to  the  smooth  internal 
muscle  of  the  eye.  A  second  or  bulbar  autonomic  system  comes  through 
the  facial  and  goes  as  the  tensor  tympani  to  the  salivary  glands.  The 
glossopharyngeus  and  vagus  belong  to  this  bulbar  autonomic  system. 
A  sacral  autonomic  system  supplies  the  organs  of  the  pelvis  and 
genitals. 

How  this  division  will  prove  out  in  clinical  work  is  yet  to  be  tested, 
but  it  has  seemed  a  necessary  hypothesis  in  codifying  the  results  of 
pharmacological  experimentation.  This  is  particularly  to  be  seen  in 
the  reactions  of  the  vegetative  nervous  system  to  certain  products  of 
the  endocrinous  glands,  i.  e.,  hormones,  and  to  certain  toxic  substances, 
notably  nicotin.  Inasmuch  as  the  vagus  constitutes  the  chief  represen- 
tative of  the  autonomic  system,  the  terminology  is  applied  to  it  more 
particularly. 

Thus  atropin  and  the  nitrites  paralyze  the  vagus  (vagoparalytic) . 
The  former  paralyzing  the  positive  or  stimulating  element  of  the 
autonomic  system,  causing  mydriasis,  loss  of  secretions;  the  latter 
paralyzing  the  negative  or  depressing  element  and  giving  rise  to 
vasodilatation.  Vagospastic  drugs,  such  as  muscarin,  pilocarpin, 
picrotoxin,  and  physostigmin,  cause  lowering  of  blood-pressure,  weak- 
ening of  the  heart  action,  bradycardia,  myosis,  increased  secretions, 
and  increased  peristalsis. 

The  chief  contrasting  activities  of  these  two  systems  are  here  shown 
in  tabular  form  as  taken  from  the  studies  of  Frohlich,  Eppinger, 
Hess,  Loewi  and  others  (see  page  84). 

A  number  of  the  products  of  the  endocrinous  system  act  as  vagotonics; 
among  these  is  cholin,  from  the  cortex  of  the  suprarenals.  Whereas 
iodothyrin,  hypophysin,  and  adrenalin  act  partly  as  sympathicotonic, 
partly  as  autonomotonic  or  vagotonic.  Iodothyrin,  or  the  related  hor- 
mone from  the  thyroid,  acts  upon  both  systems  to  produce  the  char- 
acteristic signs  of  exophthalmic  goitre  (see  later). 

The  active  principle  of  the  hypophysis  acts  upon  both  systems  to 
produce  pictures  of  hypo-  or  hyperpituitarism  or  a  mixture  of  both, 
dyspituitarism.  Adrenalin,  from  the  inner  part  of  the  adrenal,  causes 
tachycardia,  increase  in  heart  action,  increase  of  blood-pressure  from 
contraction  of  the  bloodvessels,  mydriasis  and  exophthalmos,  paresis 
and  anemia  of  the  respiratory  and  stomach  tracts,  increase  of  sphincter 
tonus  and  of  certain  glands,  mobilization  in  the  carbohydrate  depots 
in  the  liver  and  muscles,  and  increase  in  its  oxidation.  The  antago- 
nistic action  of  the  sympathicotropic  adrenalin  and  the  vagotropic 
pilocarpin  shows  itself  in  that  adrenalin  can  counteract  a  pilocarpin 
eosinophilia  and  pilocarpin  an  adrenal  glycosuria.  Other  remark- 
able opposing  reactions  are  known  showing  the  striking  antagonistic 
physiological  possibilities  of  the  vegetative  system. 


84 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


■Me 


a  3 


CD 

M 

2i 

'ri 

:2; 

fl 

o3 

r! 

U- 

a 

>! 

(3 

{■i 

ri 

^ 

O 

OPO 

■  XX 


X 


MS  ^xxx 


3 

y 

> 

> 

o 

a 

J  ^  ^  X  M  ><  ^  ^  I  ^  ^  ^ 
^g  g^^^  g  g^§  g  § 


^^ 


c  c 


C  :ti 


m    m    «  *  r^  •  r3  fr^  -3  '-+3  *+3    2-3 

S  c  c  -^  -5  a  ,9  -e  -^  S  ° 


t-iaQ02MhHf-(Cca}cKOcca2p5O 


13 
1 

■    'g 
pi- 

03 

c!   ::;:.:   :   

_o 

^ .!_( 

^   .:::::;:::.   : 

hH 

'8- 

03 

c 

I 

c 

j 

C 
C 

"m    n 

Pit: 
1  £ 

_o 

o 
O 

o 

•  a 

■  o 

•  js 
:  g 

■  M 

Inhibition 

Stimulation 

Stimulation 

Increase 

Increase 

Increase 

Stimulation 

Stimulation 

Spasm 

Contraction 

Stimulation 

Stimulation 

a 
a  o 
o  -^ 

13    o 

Xi 

.2 

1 
Q 

a) 


li  ii  '^  T)  !> 
m  "o  Cj  S  o  tj 
^    2    3^3  ^    O 


03  Mis 

(D  0    <D  ,. 

m  ^  tc    CO  ^ 

^  O    P  > 


fcaO^  ^ 


S    O  ^  'S    - 

>3S  2^ 


^-  c  —  ■ " 
-^  a        ' 

rf    o  c"  ■' 

C    g  0) 


-3    t,'   03  _  --.    03  .5 


M- 


d     --^     O     CQ 

o  1^  0)  a^ 
-1^   ft  to  +; 


, ,    .  .         ._._^.-     -     Jo3ot<tO[Cto2'-5J:l!^do 

a-;?^T;'^o3^o-::;o^:^a>Joo3o3o3o3goa=Sc3i: 
ccPOOaiOPQajOwOaj^WHOOOOfEOfKOfiipq 


^ 

."" 

3 

it! 

ffi 

03 

d 

till 

,_^ 

o 
-1^ 

0 

o 

"> 

a 

0 

(U 

> 

> 

a 

fi 

o 

•^ 

fl 

t^ 

o, 

Tf 

o 

tn 

m 

m 

>> 

03 

n 

3 

3 

-! 

(1) 

0) 

<li 

!h 

a 

aj  -1^  -|j  ^ 

03 
O 

w 

d 

^ 

o 

oi 

03 

T3 

^ 

<!j 

C 

CC 

o  o  .ii  s  -^5  c  ■ 


as 


.2  -S 


o  o  S 


O  5    O    O  73  VS  •  r3    0 


,_,  fl       el       c3 

.S-S.2.2.2.2  o  o '^  .2 '^  .2  "-JS 

>^'3    >,o3    o3    o3."t;.'S    P    ^    ?^    o3    i^i  -a  Tl 

03 -S  3l3'3'a3'S'S  oIj  o"53  o'S'S 


^ 

» 

CO       • 

0.2   : 

d 

Q 

>-.     . 

^  "S     ! 

;-g 

g 

03      ■ 

h     ■ 

d^   : 

d 

Ph 

Ph     • 

OP    ■ 

Q>  <D  «    « 

CO  ^    to  ^    to    2    2 


d  d 


'r^     !  3  ^ 


^'d  ^]d 


S^  S  g  „ 


C    m    o3 


^5        -OlP^PHPPHPPHp^PiPHMM 


l-H  >  *^  '^ 

f-H  HH  I — I  I  I 


HfJ 


jd^-d  ^HH^i 


HHH 


.S  .S   o   o   o 


^        Oj 

»  X  22  rd  o 


fH  TS 


e  fl  d   d  ^ 

H    o    o    o    (-^ 


3  ^  ^;s^ 


•d  !-§    03    03    03  '-§ 
-   ^    X    !<!    X  ^ 


O      '  O  O  CO  02 


,  r^    r^    r*;    >^    n 

CtO  ^y  C  '•^  C  *^  03  C3  ri  •r-^ 
•J3  0  ri  •-!  ri  •-  '^  li  OJ  Oj  -S 
ajP^PnPPHPt^P^PiP^^S 


^^ 


gd- 

ri  ri 
"S  03 
O  "S 

OPh 


.    (D 

d  -g 


"  ft  ^  " 

ri    "  ft  c3 

d    M  S    f^ 
O    3    OJ    O 

OcoWO 


EY^E  SYMPATHETIC  85 

Inasmuch  as  this  system  is  very  markedly  under  psychical  influences, 
particularly  of  the  aft'ects,  its  relations  to  what  is  known  as  aft'ectivity 
and  ambivalence  in  psychoanalytic  literature  is  of  far-reaching  impor- 
tance.    (See  chapters  on  Psychoneuroses  and  Psychoses.) 

The  accompanying  table  shows  most  strikingly  the  material  which 
has  in  part  led  to  the  hypothesis  of  an  autonomic  and  a  sympathetic 
partition  of  the  vegetative  nervous  system. 

Special  Pathology. — Eye  Sympathetic. — The  ciliary,  pupillary  sphincter 
and  dilator  muscles,  Miiller's  orbital  muscles,  and  the  tear  glands  are  all 
vegetative  organs  of  the  eye  which  are  innervated  in  part  by  autonomic 
and  in  part  by  sympathetic  fibers.  The  pupillary  innervation  is  of 
special  moment.  The  nucleus  of  the  dilator  sympathetic  fibers  is  the 
cilio-spinal  center  in  D  1-3.  These  fibers  pass  through  to  the  superior 
cervical  ganglion  where  a  synapsis  is  made.  Here  fibers  pass  to  the 
Gasserian  ganglion,  join  with  the  trigeminus  (1),  and  in  the  long 
ciliary  nerves  pass  to  the  vessels,  dilator  pupillse,  and  to  Miiller's 
muscle,  which  pushes  the  eyeball  forward.  An  autonomic  pathway 
(sphincter)  passes  by  means  of  the  oculomotorius  and  ciliary  ganglion. 
Connections  with  the  cerebrospinal  axis  are  many.  The  central 
origin  of  the  precellular  fibers  of  the  smooth  ciliary  muscle  of  accom- 
modation of  the  iris  sphincter  is  not  certainly  fixed.     (See  Oculomotor.) 

Several  important  clinical  conditions  depend  upon  the  complicated 
pupillary  innervation,  the  chief  of  which  are:  (1)  absolutely  stiff 
pupils,  (2)  Argyll-Robertson  pupil,  (3)  sympathetic  paralysis,  (4) 
variations  and  deformities  in  pupils. 

1.  In  the  first  all  automatic  stimuli  to  the  iris  muscle  are  inoperative 
with  the  exception  of  the  sympathetic,  which  of  itself  has  a  minimal 
action.  The  pupils  are  dilated  and  distorted.  Since  the  ciliary 
ganglion  serves  for  autonomic  tonic  activity,  any  disturbance  of  the 
ganglion  gives  rise  to  great  dilatation,  which  is  more  marked  than  is 
produced  by  nuclear  or  peripheral  lesions  of  the  oculomotor.  Absolute 
rigidity  is  seen  particularly  in  fainting,  high  grades  of  anxiety  or  fear, 
frequently  in  hysteria,  in  most  epileptic  convulsive  attacks,  and  in 
central  cerebrospinal  syphilis.  Pupillary  inequalities  are  frequent 
in  the  psychoneuroses  and  such  anomalies  have  special  significance  in 
the  study  of  the  repressions  of  unconscious  material. 

2.  The  Argyll-Robertson  pupil  is  a  complicated  phenomenon.  It 
has  already  been  described.  (See  Examination.)  It  is  an  extremely 
common  accident  in  cerebral  syphilis,  as  seen  in  tabes  and  paresis 
particularly.  It  is  occasionally  found  in  extreme  alcoholism  (Korsakow 
particularly),  and  occurs  from  rare  and  isolated  lesions  of  the  corpora 
quadrigemina.  In  apes,  Karplus  and  Kreidl  have  shown  that  a 
severance  of  commissural  association  fibers  passing  in  the  arm  of  the 
anterior  corpora  quadrigemina  to  the  anterolateral  border  of  the 
anterior  corpus  will  cause  a  bilateral  reflex  pupillary  rigidity  with 
retention  of  pupillary  activity  for  accommodation,  convergence  and 
psychical  stimuli.     Chronic  meningeal  exudates  in  syphilis  pressing 


86  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

upon  these  fibers  may  account  for  the  frequency  of  this  symptom  in 
tabes  and  paresis.  Explanations  are  numerous,  however,  and  may  be 
consulted  in  the  literature.  (Willbrand  and  Saenger,  Die  Neurologie 
des  Auges.) 

3.  Sympathetic  paralysis,  or  Horner's  syndrome,  is  characterized 
by  retraction  of  the  bulb,  narrowing  of  the  palpebral  fissure,  dropping 
of  the  upper  and  raising  of  the  lower  lid  and  myosis,  with  conservation 
of  the  psychical  and  light  reflexes  of  the  pupil. 


To  non-striated  part  of  ^ 
Lev.  Palp.  Sup. 


Medulla 


Cilio-sjjinal  Centre- 


Upper  Lid 


Dilator  Pupillce 
Long  Ciliary  Nerves 

Gasserian  Ganglion 

^Muscle  of  SlilUer 
{non-striated) 
To  Hypoglossal  Nerve 
Sweat  Fibres 
Vaso-motor  i& 
dilator  Fibres 


Middle  Cervical  Ganglion 


Inferior  Cervical  Ganglion 


Fig.  28.- 


-Diagram   of   course   of  oculo-pupillary  fibers   of   cervical  sympathetic. 
(Stewart.) 


Topographically  the  picture  results  from  pressure  on  the  sympathetic 
fibers  (goitre),  a  lesion  of  the  cervicodorsal  cord  (hematomyelia), 
gliosis  (syringomyelia),  myelitis,  especially  of  the  upper  dorsal  region 
(Dejerine,  Klumpke — Budge's  centers),  thrombosis  of  the  posterior 
inferior  cerebellar  artery,  cervicodorsal  radiculitis,  and  in  certain 
hysterical  conversions,  compulsive  tics,  and  psychotic  projections. 

Tests  with  cocain  and  adrenalin  are  of  value  in  determining  the 
sympathicotropic  activity,  a  2  per  cent,  cocain  solution  stimulating  the 


TEAR,  MUCOUS  AND  SALIVARY  GLANDS  87 

dilator  fibers.  A  failure  to  cause  mydriasis  is  evidence  of  weakness 
of  the  s^'nipathetic.  Where  such  a  paresis-producinji;  lesion  maj^  lie, 
pre-  or  postganglionic,  above  or  below  tlie  sui)erior  cervical  ganglion, 
can  be  determined  by  the  use  of  a  1  per  cent,  solution  of  the  sympathico- 
tonic hormone  adrenalin.  Six  drops  in  five  minutes  normally  causes 
no  action.  If  after  fifteen  minutes,  however,  there  is  a  marked  dilata- 
tion the  lesion  is  postganglionic.  Adrenalin  mydriasis  is  frequently 
present  in  anterior  and  middle  fossa  disturbances  (orbital  disease, 
fracture  of  base).  This  is  through  the  activity  of  the  sympathetic 
fibers  of  the  carotid  plexus  which  joins  with  the  trigeminus  to  the 
Gasserian  ganglion.  Thus  a  combination  of  disturbances  of  the 
supraorbital,  with  adrenalin  mydriasis  of  the  postganglionic  sympa- 
thetic paralysis,  may  give  important  evidence  as  to  the  localization 
of  a  fissure,  tumor,  or  fracture  of  the  base  of  the  skull.  Double-sided 
adrenalin  mydriasis  (Lowi's  reaction)  is  also  seen  in  hyperthyroidism, 
pancreatic  diabetes,  and  in  increased  irritability  of  the  sympathetic 
nervous  system  in  general. 

Vagotonic  reactions  give  rise  to  accommodation  cramps  with 
lachrymation  which  may  be  diminished  by  atropin.  In  youth  when 
vagotonia  is  more  pronounced,  atropin  acts  less  protractedly  than  in 
older  people,  and  pilocarpin  in  the  eye  may  cause  von  Graef  e's  symptom 
as  a  sign  of  an  increase  in  the  tonus  of  the  autonomic  levator  palpebrse. 

Tear  Glands. — These  are  autonomically  innervated  through  the 
superior  cervical  ganglion  and  sympathetically  through  the  spheno- 
palatine ganglion.  Irritation  of  the  neck  sympathetics  causes  increase, 
paresis  of  the  same,  diminution  in  the  secretions.  The  postcellular 
branches  of  the  neck  ganglia,  secretory  or  vasomotor  fibers,  pass  in 
the  internal  carotid  plexus  reaching  the  glands  either  by  the  way  of  the 
ophthalmic  plexus  or  through  the  cavernous  plexus  and  the  lachrymal 
sensory  branch  of  the  trigeminus. 

Mucous  and  Salivary  Glands.— The  vegetative  control  of  thesejis 
exercised  through  the  sphenopalatine,  otic,  submaxillary,  and  sub- 
lingual glands.  The  sphenopalatine  sends  only  autonomic  vasodilator 
fibers  through  the  posterior  nasal  nerves  to  the  mucous  membrane 
of  the  nose;  sympathetic  vasoconstrictor  fibers  come  from  the  cervical 
sympathetics. 

The  parotid  gland  has  both  a  sympathetic  and  autonomic  supply, 
the  former  from  the  cervical  sympathetic,  the  latter  through  the  otic 
ganglion.  The  small  superficial  petrosal  is  its  viator  or  precellular 
root,  the  auriculotemporalis  of  the  trigeminus  is  its  postcellular 
branch.  The  autonomic  bulbar  center  is  Kohnstamm's  nucleus 
salivatorius  inferior.  Autonomic  stimulation  delivers  a  different  type 
of  secretion  from  sympathetic  stimulation.  Lesions  of  the  tympanic  in 
the  mastoid  operation  give  rise  to  parotid  disturbance,  and  may  be 
looked  for  in  middle-ear  disease.  The  submaxillary  and  sublingual 
glands  have  a  sympathetic  and  an  autonomic  supply.  The  latter 
which  causes  vasodilator  and  secretory  stimulating  effects  has  its 


88  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

autonomic  bulbar  center  in  Kohnstamm's  superior  salivatory  nucleus, 
its  rami  communicans  albi  in  the  chorda  tympani  and  through  the 
lingual  to  the  gland.  The  sympathetic  vasoconstriction  and  secretory 
stimulating  fibers  are  derived  from  the  cervical  sympathetic.  Auto- 
nomic stimulation  causes  the  full,  thin,  watery,  salty  secretion,  cut  off 
by  atropin;  the  sympathetic  the  scanty,  viscous  (organic  constituent) 
secretion  acted  upon  by  cocain,  cholin,  adrenalin .  Xerostomia  (Hadden) , 
xerostomia  senilis,  xerophobia,  dryness  of  stage  and  other  types  of 


Fig.  29. — The  innervation  of  the  salivary  glands:  glp,  parotid;  gsm,  sub-maxillary; 
gsl,  subungual;  ggs,  Gasserian  ganglion;  nl,  lingual  nerve;  nm,  mandibular  nerve; 
nVII,  facial  nerve  nucleus;  cht,  chorda  tympani;  VII,  facial  nerve;  IX,  glossopharyn- 
geal nerve;  ns,  nucleus  salivatorius;  gp,  petrosal  ganglion;  s,  sympathetic;  rs,  sym- 
pathetic branches;  gs,  submaxillary  ganglion;  nh,  hypoglossal  nerve;  re,  ramus  com- 
municans.    (Bechterew.) 


fright,  as  in  marked  depressions,  etc.,  are  among  the  disorders  of  the 
secretions  of  these  glands  of  neurological  and  psychiatric  interest. 
Cortical,  glossopharyngeal,  and  trigeminal  associations  are  the  basis 
for  reflex  stimulation  of  the  glands. 

Neck  Sympathetic. — The  superior  cervical  sympathetic  supplies, 
through  the  internal  carotid  nerve  and  the  internal  carotid  plexus, 
the  dilator  of  the  pupils,  Miiller's  muscle,  tear,  parotid,  and  maxillary 
and  lingual  glands,  the  pilomotors,  vasoconstrictors,  and  sweat  glands 
of  the  face. 


NECK  AND  ESOPHAGUS 


89 


The  clinical  picture  of  loss  of  function  of  the  cervical  sympathetic 
through  pressure  or  trauma  are  myosis  of  homolateral  pupil  with  retained 
reflexes,  narrowing  of  the  homolateral  palpebral  fissure,  enophthalmos, 
hyperemia  of  the  homolateral  side  of  the  skin  of  the  face  and  head, 
and  transitory  anidrosis  of  the  same  side.  The  motor  lesions 
occur  in  the  minor,  the  others  in  addition  in  deeper  seated  lesions. 

The  vagus  like  the  oculomotor, 
glossopharyngeal  and  facial  is  a 
mixed  nerve  and  contains  motor, 
sensory  and  autonomic  fibers.  The 
somatomotor  nucleus  is  the  nucleus 
ambiguus;  the  nucleus  solitarius 
the  sensory  nucleus;  the  visceral 
nucleus  for  the  heart,  lung,  and 
digestive  systems  is  the  nucleus  dor- 
salis  vagi.  All  three  sets  of  fibers 
travel  through  the  jugular  and 
nodosus  ganglia  to  form  the  vagus. 
The  two  ganglia  point  to  two  nerves 
phylogenetically :  (a)  The  pure  motor 
branches  are  the  rami  pharyngeus; 
(6)  the  pure  sensory  branches  are 
the  meningeal  and  superior  laryn- 
geal; (c)  the  mixed  motor-sensory- 
visceral  is  the  recurrent  laryngeal 
sending  motor  fibers  to  the  larynx, 
receiving  sensory  fibers  from  the 
trachea  and  the  visceral  fibers  sup- 
plying the  heart,  aorta,  and  vessels 
of  the  larynx;  (d)  the  purely  visceral 
branches  pass  to  the  digestive  tract, 
the  heart,  the  liver,  and  the  lungs. 

Only  the  visceral  branches  will  be 
taken  up  here,  the  motor  and  sen- 
sory being  discussed  later  under  the 
cranial  nerves. 

Esophagus. — The  entire  digestive 
tract  is  served  by  the  sympathetic 
(narrow  sense),  whereas  the  vagus 
(autonomic)  only  supplies  the  lower 

two-thirds  of  the  esophagus,  the  stomach,  and  the  intestines  to  the 
descending  colon.  The  combined  action  is  stimulating  (autonomic) 
and  depressing  (sympathetic),  which  actions  are  apparently  reversed 
in  the  case  of  the  heart  muscle.  Local  ganglion  cells  seem  to  regulate 
the  motor  functions.  Tactile,  thermal,  and  chemical  stimuli  are 
apparently  unresponded  to.  Deep  pressure  sensibility  is  present,  but 
the  pathways  are  not  definitely  located. 


Fig.  30. — Schematic  arrangement  of 
cardiac  nerves:  nx,  chief  motor  nucleus; 
X,  vagus  nerve;  gs,  stellate  ganglion; 
Is,  superior  laryngeal  nerve;  s,  sympa- 
thetic; pis,  solar  plexus;  pic,  cardiac 
plexus;  1,  upper  inner  branch  to  heart; 
2,  accelerator;  .3,  internal  inferior 
branch;  4,  upper  and  inferior  external 
branch;  5,  Ansa  Vieussenii.  (Bech- 
terew.) 


90  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

Stomach  and  Intestines. — Local  ganglion  cells  are  very  frequent  and 
are  to  be  sharply  distinguished  from  the  sympathetic  structures. 
They  serve  largely  for  the  motor  functions.  The  stomach  is  strongly 
under  associative  relations  with  the  sight,  hearing  and  smell  areas 
and  its  affect  (psychical)  reactivity  is  extremely  intimate.  Ordinary 
sensibility  to  tactile,  chemical,  and  thermal  stimuli  are  also  lacking 
here,  but  deep  sensibility  fibers  are  present  and  carry  pressure  stimuli 
(pain,  colic,  crises).  The  pathways  used  for  these  are  not  known. 
It  has  been  thought  that  the  pathways  here  involved  passed  through 
the  splanchnics  (sympathetic).     Eppinger  and  Hess  have  seemed  to 


Fig.  31. — Innervation  of  the  mechanism  of  swallowing:  Sn,  substantia  nigra;  Vm, 
motor  nucleus  of  the  trigeminus ;  Vs,  sensory  root  of  the  trigeminus ;  IXm,  motor  nucleus 
of  the  glossopharyngeus ;  XII,  nucleus  of  the  hypoglossus;  Xs,  sensory  nucleus  of  the 
vagus;  pm,  soft  palate;  aps,  palatal  vault;  app,  pharyngeal  vault.     (Bechterew.) 

show  that  in  laryngeal,  bronchial,  esophageal,  gastric  and  intestinal, 
genital  and  rectal  crises,  the  autonomic  system  only  is  involved; 
the  vagus  bulbar  autonomic  for  the  upper  types,  the  pelvic  autonomic 
series  for  the  lower  ones.  Seen  from  another  angle,  vagus  and 
splanchnic  (?)  crises  are  to  be  distinguished.  The  former  without 
pain,  but  with  nausea,  hypersecretion,  and  vomiting,  the  latter  with 
pain  and  hyperesthetic  skin  zones,  and  increased  epigastric  and 
abdominal  reflexes. 

The  local  topographical  diagnosis  and  the  physiological  understand- 
ing  of   nervous   dyspepsias,   the   motility   and   secretory   anomalies 


STOMACH  AND  INTESTINES 


91 


(achylia,  hypersecretion,  hyperacidity),  changes  induced  in  Addison's 
disease  and  in  exophthahnic  goitre  and  in  all  of  which  sympathetic 
(psychical)  influences  play  a  large  role  is  as  yet  not  well  grasped. 

Individual  and  social  adjustment  to  spiritual,  i.  e.,  psychical  factors, 
seems  to  influence  them  much  more  effectually  than  measures  addressed 


Fig,  32 


Fig.  32. — Scheme"*  of  stomach  innervation:  a,  b,  ganglia  in  walls  of  the  stomach; 
nXs,  sensory  nucleus  of  the  vagus;  nXm,  motor  root  of  the  vagus;  ns,  splanchnic. 
(Bechterew.) 

Fig.  33. — -Scheme  of  intestinal  innervation:  in,  small  intestine;  r,  lower  end  of  the 
large  intestine;  pis,  celiac  plexus;  plh,  hypogastric  plexus;  spl,  splanchnic;  c,c,  spinal 
center  of  intestinal  movements;  X,  vagus;  nXm,  motor  nucleus  of  the  vagus;  nXs, 
sensory  nucleus  of  the  vagus.     (Bechterew.) 


to  modify  the  perverted  chemisms  and  motility,  especially  at  the 
beginning  of  these  disorders.  There  is  little  doubt  that  long  continued 
psychical  disturbances  which  cause  very  pronounced  secretory  and 
motor  anomalies  may  ultimately  induce  definite  organic  changes. 
Many  visceroptoses  are  of  this  type.  The  relaxation  is  due  to  ir- 
regularities in  the  reciprocal   innervation   of  the   sympathetic   and 


92  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

autonomic  fibers,  induced  in  many  instances  through  psychical 
depression.     Possibly  toxic  factors  are  important. 

The  vagus,  by  way  of  the  solar  ganglion,  stimulating  the  sympathetic, 
depresses  the  peristalsis  and  secretions  of  the  intestines.  The  intestinal 
movements,  however,  may  take  place  independent  of  either.  The 
tactile-mechanism-reflexes  are  continuously  active;  chemical  reflexes 
are  operative  during  the  passage  of  absorptive  material.  Each  have 
their  sympathetic  and  autonomic  pathways — working  independently 
one  of  the  other.  The  chief  psycho-reflex  pathways  seem  to  act 
through  the  vagus;  thus  at  the  upper  end  such  psycho-reflex  activities 
show  increase  of  secretion  with  appetite,  loss  of  secretion  with  worry, 
fear,  and,  acting  on  the  autonomic  pelvic  arc,  the  various  constipations 
and  diarrheas  so  frequently  of  psychic  origin;  the  anal  erotic  and 
anxiety  neurosis  phenomena  so  well  elaborated  by  Freud  being  among 
them.  Purely  sympathetic  disturbances  with  increased  peristalsis 
and  serous  fluid  may  result  from  loss  of  function  of  the  splanchnics, 
either  as  a  vital  reaction  to  cutting,  trauma,  etc.,  or  to  psychical 
influences,  as  from  shock,  emotion,  fear,  and  desire. 

The  great  importance  of  the  autonomic  and  sympathetic  control 
factors  on  the  vessels  of  the  abdominal  cavity  and  organs  cannot  be 
more  than  mentioned.  Here  the  peripheral  vascular  regulations  are 
in  direct  contrast  with  the  abdominal  ones,  and  hence  the  purely 
mechanical  and  vital  process  of  adjustment  of  blood-pressure  regulation 
takes  place.  The  interpretation  of  the  phenomena  of  shock  must 
come  about  through  a  study  of  these  factors,  but  such  cannot  be  taken 
up  here.^ 

Another  feature  of  activity  of  the  vegetative  nervous  system  concerns 
itself  with  the  gastro-intestinal  ferments,  and  the  specific  secretions 
or  hormones  (gastrins,  gastrosecretine,  enterokinases  of  the  various 
authors).  Many  hormones  of  the  endocrinous  glands  are  thought  to 
influence  the  gastro-intestinal  functions.  The  diarrhea  of  exophthal- 
mic goitre  (thyreoglobulin)  is  adduced  as  a  classical  example  of  this 
influence.  The  stomach  mucous  membrane  contains  a  hypothetical 
hormone  which  acts  upon  the  activity  of  peristalsis  (peristalsis  hor- 
mone), through  the  sympathetic  pathways.  Hormonol  as  a  definite 
substance  has  entered  the  therapeutic  field  of  neurology  and  promises 
much  material  for  speculation  and  interpretation  at  least.  Direct 
indications  are  slowly  crystallizing. 

Rectum. — The  chief  innervation  here  is  through  the  hemorrhoidal 
plexus  and  the  inferior  mesenteric.  Here  both  autonomic  and  cere- 
brospinal influences  are  active.  Voluntary  muscle  activities  play  a 
large  role  in  defecation,  the  grade  of  tension  in  the  rectum,  however, 
is  registered  by  the  autonomic  system,  which  is  responsible  for  the 
original  impulses,  after  which  voluntary  and  involuntary  activities  are 
operative.     The  spinal  autonomic  center  is  located  in  the  lumbo- 

1  Consult  Crile,  Study  of  the  Emotions,  Philadelphia,  1915. 


RECTUM 


93 


sacral  spinal  segments.  The  cortical  association  connections  are 
thought  by  Bechterew  to  be  in  the  sigmoid  gyrus.  Frontal  association 
pathways  are  also  present,  interference  with  which  causes  involuntary 
defecation,  as  with  frontal  tumor,  general  paresis,  epileptiform  con- 
vulsion, profound  stupor,  emotional  loss  of  control,  etc. 

Interruption  of  spinal  pathways  may  cause  obstipation  or  diarrhea 
(tabes,    poliomyelitis,    multiple    sclerosis,    tumor,    syphilis    of    cord, 


Fig.  .34. — Scheme  of  pelvic  innervation:  eg,  spinal  genital  center;  \n,  first  lumbar; 
s,  sympathetic;  ct,  conus;  iUi,  ileohypogastric;  gmi,  inferior  mesenteric  ganglion;  ghp, 
hypogastric  ganglion ;  pZ/i,  hypogastric  plexus;  g/im,  hemorrhoidal  ganglion;  n/i  (above), 
hypogastric  nerve;  ne,  sacral  nerve;  s,  erigens;  npc,  common  pudendal;  nh  (below), 
hemorrhoidal  nerve;  ndp,  dorsalis  penis;  njpp,  deep  perineal;  vu,  bladder;  scr,  scrotum. 
(Bechterew.) 

hematomyelia,  syringomyelia,  etc.).  Here  deep  sensibility  conducting 
fibers — autonomic  and  cerebrospinal — are  interfered  with  and  the  auto- 
nomic reflexes  fail  to  establish  the  psychical  connections  either  for 
compulsion  (discharge)  or  control  of  sphincter  (retention).  The  anal 
reflex  here  is  of  great  localizing  value,  its  positive  appearance  ruling 
out  disease  of  the  lower  sacral  and  coccygeal  segments.  Lesions  of  the 
cervical  or  dorsal  cord  interfere  with  the  voluntary  activities  of  the 
abdominal  muscles  in  defecation,  while  lesions  of  the  lower  lumbar 


94  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

cord  cause  changes  in  the  voluntary  sphincters.  In  sacra]  lesions,  with 
involvement  of  the  external  sphincter  nucleus,  the  anus  remains  wider 
open,  not  so  sharply  corrugated,  not  as  vigorous  in  closing  and  there 
is  loss  of  the  anal  reflex.  Notwithstanding  the  loss  of  the  voluntary 
sphincter,  autonomic  closure  is  possible.  There  is  therefore  no  single 
defecation  center  in  the  lower  cord. 

Severe  constipation  may  be  of  purely  autonomic  origin.  Reac- 
tions occur  at  physico-chemical  levels,  such  as  loss  of  water;  at  vital 
levels,  i.  e.,  pain  from  kidneys,  gall-bladder,  peritonitis,  chronic  ap- 
pendicitis, hemorrhoids;  or  at  purely  psychical  levels,  where  phantasy, 
infantile  pleasure  motives,  may  play  a  large  role,  i.  e.,  anal  erotic  in 
displacement  of  affects,  birth  phantasies,  etc.  Birth  phantasies  which 
deal  with  feces,  and  which  are  concealed  behind  constipations  and 
diarrheas  are  very  frequent  among  psychoneurotics  and  psychotics, 
particularly  in  schizophrenics,  q.  v.  The  anatomical  pathways  which 
make  such  relationships  comprehensible  exist  in  the  autonomic  fibers. 
The  pelvic  autonomic "  and  the  cervical  autonomic,  principally  the 
vagus,  are  essentially  the  same  system,  and  having  intimate  anatomic 
relation,  they  thus  afford  an  anatomical  substratum  for  the  general 
deductions  of  the  close  relation  of  the  reproductive  instincts  with  the 
vagus,  anxiety  states  thereby  coming  to  represent  repressed  libido 
strivings.  Constipation  as  a  correlate  of  miserliness  is  an  instance  in 
point,  and  is  more  fully  discussed  in  the  chapters  on  the  psycho- 
neuroses. 

Vagotonic  manifestations  within  the  gastro-intestinal  tract  are  of 
considerable  importance,  although  as  yet  far  from  being  definitely 
analyzed.  Pilocarpin  and  physostigmin  increase  them,  whereas 
adrenalin  and  atropin  diminish  them.  In  vagotonic  individuals 
there  are  increased  esophageal  cardiac  spasms,  tendency  to  increased 
salivation  and  to  increased  secretions  from  the  nose  and  eyes.  There 
is  slowness  in  the  peristalsis,  as  shown  by  radioscopic  examination, 
due  to  increased  muscular  tonus.  This  latter  causes  the  stomach 
form  of  hyperkinetic  motility  gastroneurosis.  It  may  arise  from 
disorder  at  the  physico-chemical  or  psychical  levels.  Hypersecretion 
and  hyperacidity  are  accompaniments  with  pylorospasm.  Certain 
cerebrospinal  levels  seem  to  be  involved  as  shown  by  the  Head  hyper- 
sensitive skin  areas.  Membranous  enteritis  or  colitis  with  mucus  and 
many  eosinophile  cells  in  the  blood  and  mucous  secretions  is  associated 
with  this  condition  summarized  as  vagotonia.  Here  psychical  influ- 
ences are  of  great  moment.  The  constipation  just  spoken  of  may  be 
arranged  in  this  vagotonic  group.  Renal  and  biliary  colics,  spasmodic 
icterus,  reflex  anuria,  eosinophilia,  and  increased  glucose  tolerance  are 
to  be  found  in  this  vagotonic  group. 

Genito-urinary  System. — Here  autonomic  and  cerebrospinal  controls 
are  in  evidence.  The  former  act  through  the  mesenteric,  hypogastric, 
and  hemorrhoidal  autonomic  sacral  ganglia,  supplying  with  non-med- 
ullated  fibers  the  involuntary  muscles  and  the  mucous  membranes, 


GENITO-URINARY  SYSTEM  95 

N.  hypogastricus  to  muscles  of  colon  and  bladder  (sphincters),  the 
plexus  cavernosus,  and  nervus  erigens  to  the  genital  vasomotors. 
The  latter  act  through  medullated  fibers  to  the  voluntary  muscles  and 
adjacent  skin  areas.  The  nervus  pudendus  communis  supplies  the 
external  sphincter  ani,  external  sphincter  vesicse,  compressor  urethrse, 
deep  perinei,  etc. 

A  series  of  autonomic  reflexes  are  here  met  with,  the  most  important 
being : 

1.  Scrotal  reflex:  Stroking  of  perineum  or  femoral  skin;  contraction 
of  dartos. 

2.  Bladder  reflex:  Stretching  or  stimulus  (mechanical,  psychical) 
of  bladder  wall;  contraction  of  bladder  (mechanical,  psychical). 

3.  Rectal  reflex:  Stretching  or  stimulus  of  rectum;  contraction  of 
rectum. 

4.  Genital  reflex:  Psychical  or  mechanical  stimulus;  erection  and 
hyperemia;  corpus  cavernosus. 

5.  Uterus  reflex:   Stretching  or  irritation  of  uterus;  contraction. 

6.  Anal  reflex:  Stretching  of  anus;  psychical;  contracture  of 
sphincter  ani. 

All  of  these  reflexes  act  through  psychical  levels  as  well  as  through 
peripheral,  i.  e.,  somatic  ones. 

Bladder. — The  general  mechanisms  of  the  bladder  pattern  after 
those  of  the  rectum  and  quite  homologous  symptoms  foUow  disturb- 
ances of  homologous  relationships  of  the  autonomic  and  spinocerebral 
pathways.  The  chief  autonomic  series  travel  in  the  sacral  vesical 
nerves  to  and  from  the  inferior  mesenteric  and  hypogastric  ganglia. 
Sympathetic  fibers  are  also  functionating  through  the  hypogastric 
to  and  from  the  inferior  mesenteric  ganglia.  Thus  the  bladder  has 
a  vegetative  mechanism  comparable  to  that  of  the  pupils. 

Emptying  of  the  bladder  follows  similar  lines  to  that  of  emptying 
of  the  rectum.  Section  of  the  cord  to  above  the  mid-dorsal  region 
brings  about  automatic  emptying.  Psychical  influences  are  here 
active  as  in  the  case  of  the  rectum — urethral  erotic  with  retention  and 
incontinence  of  purely  psychical  character.  These  are  discussed  under 
psychoneuroses,  whereas  the  more  mechanical,  neurological  features 
are  taken  up  under  diseases  of  the  spinal  cord. 

Sexual  Organs.— 'Autonomic  and  sympathetic  supplies  are  present. 
The  former  carry  stimuli  through  the  nervi  erigentes  from  the  sacral 
cord  producing  vasodilatation  and  erection,  turgor  in  the  female, 
nipple  erection,  etc.  The  latter  carry  stimuli  through  the  hypogastric 
nerve  to  cause  vasoconstriction  and  contraction  of  the  unstriped 
musculature  of  the  sexual  glands  and  discharge  channels.  In  the 
sexual  act,  orgasm,  the  sexual  desire,  erection  and  ejaculation  may 
show  separate  mechanisms.  At  the  physico-chemical  level  the  con- 
cretization  of  sexual  desire  usually  reaches  an  active  adult  stage  with  the 
onset  of  puberty.  It  is  assumed  that  chemical  stimuli — hormones — 
act  at  this  level  to  cause  tension — tumescence — within  the  organs  them- 


96  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

selves,  and  also  possibly  working  upon  higher  level  nervous  structures 
cause  an  increase  in  vital  and  psychical  tension,  thereby  causing 
increased  sensitization  to  sensory  contacts  and  to  mental  stimuli. 
Thus  the  love  impulse  springs  up  at  a  touch  or  under  the  influence  of  a 
symbolic  expression,  as  in  poetry,  or  other  artistic  creation. 

The  whole  impulse  of  life  and  of  the  principle  of  race-preservation, 
i.  e.,  immortality,  is  bound  up  in  the  instinct  of  reproduction.  The 
energy  of  this  instinct  has  been  termed  libido  by  various  writers,  by 
others  the  word  is  used  in  a  wider  sense,  as  synonymous  with  the  life 
energy  wherein  one  can  distinguish  a  nutritive  and  a  sexual  component. 

Certain  hints  obtained  from  the  study  of  the  processes  of  reproduc- 
tion in  lower  organisms — protozoa,  protophyta — tend  to  show  that 
continuance  of  the  life  of  the  individual,  and  of  the  species,  has  been 
obtained  through  a  sacrifice  of  the  ego,  i.  e.,  purely  individual  reproduc- 
tion gave  way  to  gametic  reproduction,  i.  e.,  to  the  principle  of  fertiliza- 
tion by  sexual  processes.  A  complete  discussion  of  this  principle  cannot 
be  given  here,  but  it  is  assumed  that  the  human  species  shows  the 
same  general  trends  with  many  more  complicated  reactions  within  all 
three  levels  of  the  nervous  system.  Hence  the  hypothesis  which 
assumes  that  both  vital  and  psychical  sexual  manifestations  may  be 
stimulated  by  purely  physico-chemical  determinants,  such  as  tension 
within  the  glands,  or  by  the  activity  of  specific  chemical  substances, 
sexual  hormones — from  both  testicles  and  ovaries — which  in  the  onto- 
genesis of  the  individual  may  serve  as  stimuli  to  the  development  of 
structure,  and  also,  given  such  structures,  may  aid  in  their  devel- 
oped functioning.  Inasmuch,  however,  as  vital  energy  acting  solely 
through  physico-chemical  processes  does  not  aft'ord  any  adequate 
explanation  for  all  and  least  of  all  for  the  most  important  of  the 
phenomena  of  evolution,  an  adequate  hypothesis  must  also  include 
similar  activities  at  higher  levels,  i.  e.,  vital  and  psychical.  The 
out-and-out  materialist  stops  at  the  lowest  levels,  the  vitalist  midway, 
the  evolutionist  argues  for  the  leadership  of  the  psychical,  but  needs 
the  interrelationship  of  all.  Psychical  impotence  with  intact  organs, 
for  instance,  is  inexplicable  on  materialistic  or  vitalistic  hypotheses. 

Seen  from  another  angle  this  vexed  subject  of  interrelationships  is 
well  illustrated  in  the  large  disease  group  of  schizophrenia  (dementia 
precox).  From  the  psychical  side  alone  some  have  endeavored  to 
explain  it  as  a  series  of  reactions  to  repressed  and  unconscious  sexual 
activities — repressed  and  unconscious  because  of  higher  cultural 
demands  and  inability  on  the  part  of  the  patient  to  sublimate  i.  e., 
employ  his  libido  in  its  numerous  useful  transformations  arrived  at 
in  the  course  of  cultural  development.  A  compromise  situation  adopts 
the  interrelatory  hypothesis  granting  a  somatic  inferiority  of  brain 
structure  (defect  or  degeneration)  of  physico-chemical  origin  (dyspitui- 
tarism  or  other  glandular  defect)  with  a  symptomatology  determined 
largely  by  symbolizations  of  the  sexuality.  The  Abderhalden  preg- 
nancy reaction — ovarian,  testicular,  hormone  changes — shows  a  pecu- 


RESPIRATORY  APPARATUS  97 

liar  activity  with  schizophrenics  pointing  to  some  disturbance  at  the 
physico-chemical  level.  This  whole  matter  is  still  speculative,  but 
affords  an  excellent  illustration  of  the  possible  value  of  thinking  in 
all  of  the  terms  proposed. 

In  the  phenomenon  of  erection  one  sees  these  principles  at  work. 
The  cerebral  or  psychical  is  the  most  frequent  source  of  origin  for  vaso- 
dilation. The  pathways  are  by  means  of  the  cord  to  the  upper  lumbar 
segments  and  by  way  of  the  erector  nerves.  In  severe  spinal  injuries 
psychical  erection  may  remain  intact.  Severe  continuous  priapism 
is  not  infrequently  of  purely  cerebral  origin,  either  organic  as  in 
encephalitis,  non-purulent  or  purlent,  syphilitic  (paresis),  or  possibly 
purely  psychical  as  in  some  manic  states,  some  schizophrenics. 

Vital  levels  respond  to  the  sensory  stimuli  of  the  skin  of  the  penis 
or  adjacent  organs,  and  the  reflex  pathways  are  made  up  of  the  spinal 
sensory  nerves,  the  second  sacral  segment  and  the  dorsalis  penis  and 
pudendis  communis  nerves  acting  through  synaptic  junctures  in  the 
sympathetic  ganglion.  Transverse  lesions  of  the  cervical  dorsal  cord 
may  also  induce  priapism.  Certain  cases  of  encephalitis  just  men- 
tioned show  cervical  cord  lesions  as  well. 

The  physico-chemical  levels  respond  to  the  tension  stimuli  from  the 
bladder,  seminal  glands,  etc.,  acting  through  the  hypogastric  plexus. 

In  ejaculation,  sympathetic  and  cerebrospinal  pathways  are  utilized. 
A  summation  of  stimuli,  acting  through  the  sympathetic,  forces  the 
threshold,  setting  free  a  peristaltic  contracture  of  the  vasa  deferentia 
with  the  accumulation  of  sexual  secretions  in  the  prostatic  portion  of 
the  urethra.  A  spinal  reflex  causes  the  contraction  of  the  bulbo- 
and  ischiocavernous  muscles  with  the  ejaculation  of  the  semen. 

Sympathetic  disturbances  are  rare,  spinal  ones  not  infrequent  in 
conus  lesions,  either  being  traumatic  or  due  to  new  growth  or  infiltrating 
disease,  tumor,  syphilis,  etc.  Ejaculation  in  coitus,  in  masturbation, 
or  in  pollution  dreams  is  usually  accompanied  by  other  autonomic 
signs,  such  as  mydriasis,  hyperidrosis,  and  cardiac  palpitation.  Pollu- 
tion dreams  have  determinants  at  all  the  levels  mentioned.  They 
are  usually  not  harmful.  When  frequent  and  evidently  pathological 
they  may  arise  from  lower  level  stimuli  (prostatic  disease,  etc.), 
but  are  more  often  of  psychical  origin — usually  accomplishing  the 
repressed  and  unconscious  wish  for  culturally  forbidden  sexual  activi- 
ties (masturbation,  homosexuality,  ffidipus  phantasy).  Hence  their 
great  frequency  in  the  psychoneuroses,  in  schizophrenia,  or  in  compul- 
sive states,  unless  some  other  type  of  symbol  carries  out  the  forbidden 
and  repressed  wish. 

Respiratory  Apparatus. — Complete  data  are  not  available  for  definite 
plotting  of  the  vegetative  nerve  physiology  in  this  region.  Autonomic 
vagus  fibers,  acting  through  the  superior  laryngeal,  tracheal,  and 
bronchial  nerves,  induce  reflex  coughing,  inflammatory  reactions  with 
increase  of  mucus,  etc.  Somatic  fibers  are  concerned  as  well.  Hys- 
terical coughing  utilizes  the  autonomic  pathways.  Asthmatic  attacks, 
7 


98 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


with  spasm  of  the  bronchi,  difficulty  in  breathing,  slowing  of  respira- 
tory phases,  emphysema,  and  eosinophilic  sputum,  are  illustrations  of 
increased  vagotonia,  hence  relieved  in  part  by  adrenalin.  Here  the 
exciting  causes  may  also  lie  at  any  of  the  three  levels.  Physico-chemical 
(parathyroid  with  tetany,  calcium  metabolism),  vital  (from  pressure 
phenomena  on  laryngeal  and  bronchial  nerves,  reflexes  from  nose), 
or  psychical  (emotions,  sexual  excitement,  repressed  sexuality).  The 
problem  in  treating  asthma  is,  therefore,  to  find  which  nerve  system 
level  is  chiefly  implicated.  Psychoanalysis  would  be  folly  for  those 
asthmatic  attacks  which  are  due,  for  instance,  to  cheesy,  tuberculous 

deposits  pressing  upon  nerve  structures 
in  the  posterior  mediastinum,  while  it 
alone  would  remedy  those  asthmas 
that  are  of  psychical  origin  solely. 
Combined  therapy — interrelational — is 
of  greatest  value. ^ 

Vascular    Apparatus. — Only    a    brief 
outline  is  possible. 

Heart.  —  Sympathetic,  autonomic, 
and  intraganglionic  mechanisms  are 
present.  The  sympathetic  pathways 
arise  from  D  1-5.  Wrisberg's  ganglion 
is  the  first  synapsis,  the  postganglionic 
fibers  passing  to  the  heart  musculature. 
The  vagus  is  active  through  three  main 
branches,  one  arising  below  the  supe- 
rior laryngeal,  a  second  from  the  recur- 
rens,  a  third  from  the  thoracic  part  of 
the  vagus.  The  deeper  layers  of  the 
heart  are  supplied  through  the  right 
vagus,  the  superficial  cardiac  plexus 
supplying  through  the  left.  The  sym- 
pathetic fibers  which  accelerate  the 
heart's  action  are  in  relation  with  the 
extra  cardiac  ganglia,  the  end  branches 
of  the  vagus  in  connection  with  the 
intracardial  ganglion,  i.  e.,  cells.  These 
intracardial  cells  are  here  assumed  to  be  visceromotor,  and  are 
thickest  at  the  origin  of  His'  bundle,  Tawara's  nodes  and  at  the 
origin  of  the  Keith-Flack  bundle.  The  activity  of  the  vagus  upon 
the  muscles  seems  to  manifest  itself  chiefly  through  the  ganglion  cells. 
The  gray  matter  of  the  midbrain  in  the  neighborhood  of  the  floor 

1  Cheyne-Stokes  respiration  is  found  in  a  great  variety  of  pathological  states  such  as 
high  cervical  myelitis,  hemorrhage  of  the  medulla,  hemorrhage  of  the  base,  tumors 
of  the  midbrain  region,  and  occasionally  in  certain  cortical  atrophies  or  hemorrhages. 
It  is  rarely  present  in  certain  hysterics.  Snorting,  barking,  coughing,  sneezing,  hic- 
coughing and  yawning  are  frequent  respiratory  affections.  They  are  for  the  most  part 
psychical,  but  not  always. 


Fig.  35. — Scheme  of  innervation 
of  breathing:  D,  diaphragm;  nf, 
phrenic  nerve;  X,  sensory  vagus 
branches  to  the  lungs;  nr,  respira- 
tory nucleus  in  medulla;  nXs,  sen- 
sory nucleus  of  the  vagus;  nrs, 
respiratory  center  in  midbrain 
region.     (Bechterew.) 


VASCULAR  APPARATUS 


99 


of  the  third  ventricle  is  thought  to  be  a  higher  coordinating  switch- 
board— the  nucleus  dorsalis  vagi,  an  end  station.  Through  this  portion 
of  the  mechanism,  psychical  influences  are  switched  in,  modifying  the 
tonus  through  emotions,  pain,  and  local  stimuli. 


Fig.  36. — Scheme  of  cardiac  innervation :  A.d, 
right  auricle;  A.s,  left  auricle;  V.d,  right  ven- 
tricle; V.s,  left  ventricle;  v.c.s,  and  v.c.i,  superior 
and  inferior  vena  cava;s.i;.c,  sinus venosus;  F.o, 
foramen  ovale;  K.F.,  Keith-Flack  sinus  node; 
A.T,  Aschoff-Tawara  auriculoventricular  node; 
Th,  Thorel's  superior  vena  cava  bundle;  Tho, 
Thorel's  intermediary  node  bundle;  W,  Wenck- 
enbach's  auriculo venous  bundle;  k.f.,  Keith- 
Flack  fibers  between  K.F.  and  F.o. ;  a.t.,  Aschoff- 
Tawara  bundle  between  A.t.  and  F.o.;  P.H, 
Paladino-His  bundle.     (Janowski.) 


Fig.  37. — Scheme  of  innervation 
of  the  vasomotors:  cv,  main  center 
of  vasomotors  in  the  medulla;  cs, 
spinal  vasomotor  centers;  s,  sym- 
pathetic; nX,  vagus  nucleus;  X, 
vagus;  nV,  trigeminus  nucleus;  plh, 
brachial  plexus;  plv,  vesical  plexus; 
pulm,  lungs;  stom,  stomach;  duod, 
duodenum;  hep,  liver;  spl,  spleen; 
ren,  kidney;  r,  rectum;  res,  bladder; 
scr,  scrotum;  pn,  penis.  (After 
Langley.) 


Bradycardia  appears  through  a  number  of  influences,  chiefly  follow- 
ing acute  infectious  intoxications,  by  increased  intracranial  pressure, 
in  hypothyroidism,  digitalis  and  allied  glucosidal  actions.  Trigeminal 
reflexes  through   the   nose,  eyes   (pressure)   may  also   cause  brady- 


100  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

cardia.  The  various  arrhythmias,  dislocations,  and  blocks  cannot 
be  discussed  here. 

The  relation  of  changes  in  or  due  to  His'  bundle  cannot  be  entered 
into  here,  although  they  may  properly  be  discussed  in  a  text-book 
on  neurology. 

Angina  pectoris,  in  some  of  its  forms  at  least,  is  due  to  autonomic 
overstimulation  whereby  vascular  cramp  states  are  brought  about; 
vagus  paralyzing  and  vasodilator  drugs,  therefore,  aid  in  overcoming 
the  condition,  particularly  in  the  vasomotor  types  of  angina.  Cardiac 
discomfort  so  frequent  in  visceral  heart  disease,  as  well  as  in  psychical 
disorders  is  carried  to  consciousness  chiefly  through  communicating 
sympathetic  branches  through  the  spinal  ganglia,  or  directly  to  the 
spinal  systems.  Head's  hyperalgesias  are  explained  in  this  manner. 
The  vagus  (autonomic)  fibers  are  not  implicated. 

Bloodvessels:  Vasomotor  Neuroses. — The  anatomy,  physiology,  and 
clinical  disturbances  of  the  bloodvessels  make  a  large  chapter  in  con- 
temporary neurology.  Cassirer  has  devoted  a  monograph  of  1000 
pages  alone  to  their  consideration.  Only  the  briefest  sketch  is  offered 
here. 

The  bloodvessels  of  the  face  are  innervated  from  the  upper  cervical 
sympathetic  fibers  passing  over  the  internal  carotid  plexus  to  the 
Gasserian  ganglion,  and  with  the  pathways  to  the  sweat  glands  pass 
with  the  sensory  fibers  of  the  face.  Those  of  the  upper  extremities 
are  supplied  chiefly  from  C5  to  D7,  mostly  leaving  by  way  of  the  D3  to 
D7  root  segments.  Those  of  the  lower  extremities  arise  from  D12 
to  L3.  In  the  spinal  axis  are  located  only  the  local  segmental  functions. 
Bulbar  centers  are  present  in  the  nucleus  dorsalis  vagi,  which  is  an 
autonomic  center  for  peripheral  vessels  as  well  as  those  of  the  intestines. 
Stimuli  in  the  bulbar  centers  tend  to  cause  contraction  of  the  peripheral 
vessels  and  dilatation  of  the  visceral  ones.  Intracranial  bloodvessels 
have  vasodilator  and  vasoconstrictor  pathways,  possibly  conveyed 
through  the  cervical  sympathetics. 

Cortical  centers  have  been  placed  in  the  frontal  areas  (Lewandowsky, 
Weber;  denied  by  Miiller  and  Glaser,  who  claim  the  midbrain  as  the 
highest  center),  from  which  the  pathways  pass  through  the  internal 
capsule,  caudate  nucleus,  thalamus,  hypothalamus,  pons,  central  gray 
of  fourth  ventricle,  oblongata,  Helweg's  triangular  bundle,  anterolateral 
bundle  to  lateral  horns — the  fibers  crossing  in  the  posterior  commissure 
(Helweg).  The  autonomic  and  sympathetic  fibers  apparently  follow 
different  pathways  from  the  cord,  the  vasodilator  autonomic  pathways 
following  the  course  of  the  sensory  roots,  the  vasoconstrictor  sym- 
pathetic by  way  of  the  anterior  roots,  the  motor  nerves,  and  the 
sympathetic  ganglion.  Thus  irritation  of  the  posterior  roots  causes 
hyperemia  (vasodilatation)  with  pain;  paralysis  of  the  same  causes 
anemia  with  anesthesia. 

Within  the  bloodvessels  themselves  ganglion  cells  are  found,  save 
perhaps  in  those  whose  vasomotors  run  in  the  spinal  nerves  (Miiller 


< 

Oh 


fl 

0) 

^ 

-^ 

S- 

'sT 

0 

0 

CA 

'>. 

r 

h 

0 

g 

T3 

^ 

D5 

r: 

Q 

p 

•i-H 

0 

f; 

CC 

w 

^ 

C5 

o 

f- 

a) 

i-j 

c 

cC 

+j 

0 

0 

fc 

03 

03 

CC 

0) 

B 

0 

Sh 

c 

-^j 

>^ 

S- 

W 

VASOMOTOR  NEUROSES  101 

and  Glaser),  and  reflexes  occur  here  exactly  as  in  all  of  the  skin  and 
tendon  reflexes  from  terminal  stimuli.  Hence  an  analysis  of  vascular 
disturbances  must  include  a  stud}'  of  the  sensory,  motor  and  central 
portions  of  the  reflex  arc — the  last  including  both  medullary  and 
corticospinal  reflex  pathways. 

VASOMOTOR  NEUROSES. 

Disturbances  of  the  peripheral  mechanisms  of  the  vasomotor 
pathways  have  been  more  completely  analyzed  than  those  occurring 
in  the  spinal,  bulbar,  thalamic,  or  cortical  portions  of  the  same.  Of 
these,  more  detailed  mention  may  be  made  of  the:  (1)  tonic  hyper- 
emias (erythromelalgia),  (2)  spastic  anemias  (pseudosclerosis,  Ray- 
naud's disease,  migraine,  intermittent  claudication),  and  (3)  vaso- 
motor irritability,  as  in  acute  angioneurotic  edema,  multiple  gangrene 
of  the  skin,  etc. 

Physiological  alterations  in  the  tonus  of  the  peripheral  vessels  are 
seen  in  sleep,  emotional  states,  active  digestion,  overexercise,  over- 
heating, in  collapse,  and  in  fatigue  states. 

1.  Tonic  Hyperemias. 

These  consist  in  prolonged  irritability  of  the  peripheral  vasomotors. 
In  certain  individuals  (sympathicotonic)  a  diminished  alkalinity  of  the 
blood  is  thought  to  bring  about  such  a  stimulation  of  the  sympathetics. 
These  tonic  hyperemias  are  seen  more  particularly  in  neuralgias,  neu- 
ritides,  in  infections,  or  toxic  erythemas,  and  reach  a  pronounced  grade 
in  the  syndrome  known  as  erythromelalgia. 

Erythromelalgia. — Two  main  trends  may  be  distinguished — those 
with  pain  as  described  by  Weir  Mitchell,  and  those  without  pain  but 
with  hyperidrosis  and  hyperalgesia  (Hess). 

Weir  Mitchell,  in  1878,  described  a  paroxysmal  disorder  of  the 
extremities  which  was  marked  by  a  painful  redness  and  swelling  of 
the  feet.  Lannois,  in  1880,  wrote  an  important  monograph  on  the 
subject,  and  Cassirer,  in  the  second  edition  of  his  Vasomotorisch- 
trophischen  Neurosen,  1912,  has  given  a  complete  description  of  the 
general  group  to  which  the  name  erythromelalgias  may  be  given.  He 
was  able  to  gather  reports  of  about  130  cases.  One  may  conclude  it 
to  be  rare.  Only  2  in  Oppenheim's  25,000  dispensary  patients  are 
reported,  while  in  Jellifi^e's  statistics  of  Starr's  dispensary  service 
of  18,000  patients  21  were  observed,  15  in  males  and  6  in  females. 
It  is  more  often  observed  in  the  later  years  of  life,  although  six-to 
ten-year-old  (Baginsky)  patients  are  recorded. 

Causes  are  difficult  to  run  down.  Thermic  influences  apparently 
play  some  role  as  exciting  agents  at  most.  Psychical  factors  may 
determine  an  attack. 

Hypothetically  erythromelalgia  is  a  pure  sympathetic  affection, 
an  angioneurosis,  due  to  prolonged  sympathetic  stimulation.     Prac- 


102  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

tically  it  shows  itself  in  combined  forms,  being  an  accompaniment 
of  spinal  disorder  (involving  the  sympathetic  cell  groups)  in  multiple 
sclerosis,  and  in  tabes;  it  may  occasionally  be  seen  in  cerebral  disorders, 
hemiplegia,  thalamic  involvement,  or  may  be  a  part  of  a  peripheral 
nerve  disorder,  accompanying  a  neuritis,  or  it  may  be  a  cause  of  or  a  part 
of  a  chronic  vascular  disease  of  an  obliterating  or  spasmodic  type.  In 
each  of  these  the  chief  action  is  directed  upon  the  sympathetics.  Thus 
a  number  of  gradations  and  variants  exist  which  are  discussed  in  the 
works  already  cited,  particularly  in  Cassirer  and  Oppenheim. 

Symptoms. — The  chief  symptoms  are  heat,  redness,  and  pain  in  the 
extremities,  either  localized  along  a  definite  nerve  distribution,  often 
following  a  root  area,  or  peripheral.  It  is  usually  intermittent,  worse 
at  night,  and  the  patient  suffers  tortures.  Heat  usually  makes  it 
worse,  so  also  does  movement,  especially  walking,  whereas  any  position 
reducing  passive  congestion,  thus  overcoming  the  tonic  hyperemia, 
affords  relief.  Severe  grades  of  the  disorder  show  a  purple  cyanotic  skin, 
with  erythema,  usually  due  to  transudation  following  stasis  from  slowed 
circulation  in  the  area  of  vasodilatation.    Hyperidrosis  may  be  present. 

Accessory  symptoms,  such  as  headache,  palpitation,  and  fainting, 
are  reactions  to  the  pain,  and  in  part  to  the  fear,  or  may  be  another 
facet  of  a  psychoneurosis  in  which  the  erythromelalgia  is  also  a  symp- 
tom. Trophic  changes  in  the  skin,  hair  and  nails  may  take  place, 
which  are  either  a  part  of  the  sympathetic  disturbance  itself  or  are 
results  common  to  the  angioneurosis,  and  a  producing  or  accompanying 
lesion — tabes,  multiple  sclerosis,  paresis,  etc. 

Course  and  Therapy. — The  outcome  depends  much  upon  the  causa- 
tion. An  erythromelalgia  due  to  spinal  changes  may  get  better  if 
these  do  (syphilis)  or  not  (tumor,  multiple  sclerosis).  The  therapy 
will  be  determined  by  the  cause.  Palliatives,  such  as  the  use  of  high 
frequency  current,  violet  rays,  cold,  antipyrin,  are  valuable  as  well. 

A  neuritic  erythromelalgia  will  improve  or  not  as  the  neuritis  does; 
similarly  an  arteriosclerotic  one;  but  it  usually  gets  worse.  The 
therapy  is  for  the  more  fundamental  condition.  A  psychically  deter- 
mined erythromelalgia,  possibly  a  hysteria,  needs  psychoanalysis. 

2.  Spastic  Anemic  Group. 

Here  the  chief  results  are  due  to  persistent  or  intermittent  vaso- 
constriction. The  syndromes  are  numerous  and  confusing,  but  among 
them  a  few  are  sufficiently  distinct  or  constant  to  be  given  diagnostic 
titles  such  as  Raynaud's  disease,  intermittent  claudication,  migraine, 
pseudosclerosis,  asphygmia  alternans,  etc.  Only  the  chief  types  can 
be  taken  up;  the  purely  tentative  nature  of  the  classification  must  be 
emphasized. 

Raynaud's  Disease. — Raynaud's  disease  is  also  known  as  symmetrical 
gangrene,  local  asphyxia.  This  syndrome,  like  the  preceding  one,  may 
be  of  many  origins.  It  may  be  psychical  (shock,  hysteria,  schizo- 
phrenia), cerebral,  or  spinal  organic  (capsular  (thalamic)  hemorrhage. 


PLATE    III 


Fig.  2 


Raynaud's  Disease,   sho^A^ing   superficial  gangrene.     (Osier.) 


VASOMOTOR  NEUROSES  ,  103 

trauma,  paresis,  multiple  sclerosis,  tabes,  syringomyelia,  tumors),  or 
peripheral  in  nerve  or  bloodvessels,  neuritis  of  all  various  etiologies, 
arteriosclerosis  either  peripheral  or  of  the  large  vessels  (aorta). 

It  may  readily  be  seen  that  from  such  a  polyetiological  viewpoint 
there  is  no  true  Raynaud's  disorder.  Hence,  Cassirer's  attempt  to 
make  true  sympathetic  types  and  those  due  to  complicating  disorders, 
such  as  the  local  gangrenes  due  to  diabetic  neuritis,  or  to  arterio- 
sclerosis, etc.  Even  this  is  difficult  to  accomplish.  Thus  a  spinal  gliosis 
(syringomyelobulbia)  may  invade  the  sympathetic  cells  in  a  given 
segment  and  bring  about  a  local  paroxysmal  gangrene.  This  is  truly 
a  sympathetic  affection,  but  it  is  to  be  considered  a  part  of  the  syringo- 
myelia just  as  much  as  it  is  to  be  considered  Raynaud's  disease,  even 
if  it  happens  to  be  the  very  beginning  symptom  of  the  syringomyelia. 

Symptoms. — The  attacks  are  paroxysmal.  The  fingers  or  toes  begin 
to  get  cold,  and  have  the  feeling  of  prickling  and  of  going  asleep.  They 
become  pale  and  waxy  from  the  vasoconstriction.  Pain  is  frequently 
felt  and  local  coldness  is  present.  An  attack  of  this  kind  may  come  and 
go  in  a  few  hours. 

More  persistent  attacks  lead  to  more  marked  grades  of  local  asphyxia, 
with  cyanosis,  or  bluish-red  discoloration  of  the  extremities.  Pain  is 
extreme.  Vesicles  may  form — the  fingers  may  even  get  bluish-black — 
and  all  gradually  disappear  after  a  few  days,  or  leave  slowly  healing 
broken  vesicles,  or  more  deeply  lying  trophic  ulcers  (protopathic 
nerve  fiber  injury).  Other  types  of  sensibility  also  suffer.  Epicritic 
touch  and  thermal  as  well  as  protopathic  pain,  thermal  and  deep 
sensibility,  may  also  be  involved.  Gangrene  is  a  severe  grade  with 
loss  of  fingers  or  finger  tips. 

Accessory  symptoms  (such  as  trophic  changes  in  the  nails,  in  hair, 
in  the  bones,  etc.),  which  are  due  to  the  different  etiological  factors, 
syringomyelia,  neuritis,  arteriosclerosis,  etc.,  need  not  be  entered  into. 
Attacks,  with  recovery,  may  persist  as  long  as  three  to  four  months. 

Treatment. — The  therapy  is  often  without  avail,  as  the  underlying 
condition  is  unmodifiable  (syringomyeha,  multiple  sclerosis,  etc.). 
As  a  rule,  however,  the  attack  subsides,  although  to  appear  again. 
Then  attention  should  be  addressed  to  the  general  health  of  the  patient, 
especially  to  emotional  features  which  produce  vascular  instability. 
Mild  massage,  local  warmth  and  Bier's  hyperemic  treatment  are  of 
value  during  the  attack,  strong  analgesics  being  necessary  for  the 
pain  at  the  time.  In  psychogenic  cases  psychotherapy  is  alone  available. 
Intermittent  Claudication. — This  is  an  angiospastic  syndrome  and 
rests  upon  a  number  of  foundations.  Clinically  it  consists  of  a  spastic 
vascular  state  with  weakness,  pain,  and  coldness  in  the  affected  region. 
In  the  majority  of  cases  it  appears  in  the  leg  or  legs.  After  the  patient 
has  walked,  perhaps  rapidly,  the  leg  or  legs  begin  to  be  fatigued,  and 
commence  to  feel  numb  and  painful  until  it  is  impossible  to  keep  up 
the  pace  or  walk  at  all.  After  a  rest,  the  patient  may  resume  his  walk 
for  a  time  free  from  distress,  but  the  state  of  pain  and  fatigue  recurs  to 
be  again  relieved  following  rest.     There  is  a  later  tendency  for  the 


104  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

state  to  recur  when  the  hmbs  are  at  rest.  Cyanosis,  coldness,  paleness 
are  accompanying  phenomena.  There  is  mild  hyperesthesia  of  the 
affected  part  but  no  other  sensory  signs.  The  chief  vessels  may  be 
pulseless.  These  should  be  tested  by  touch  and  the  eye  aided  by  the 
sphygmograph. 

The  chief  sites  are  the  vessels  of  the  legs  but  the  arms  may  be 
involved.  Any  muscular  group  may  show  the  symptoms.  Lumbago- 
like forms  occur  in  the  back  muscles.  The  vessels  of  the  intestines, 
internal  organs,  brain,  and  spinal  cord  may  be  involved. 

The  chief  lesion  is  arteriosclerosis,  but  others  are  operative.  The 
arteriosclerosis  itself  may  be  secondary  to  syphilis,  alcoholism,  to 
chronic  nicotine  poisoning.^ 

Oppenheim  has  called  attention  to  the  frequency  with  which  these 
arterial  changes  are  found  in  Russian  Jews.  This  disorder  is  mostly 
confined  to  the  men  of  this  people.  Here  flat  foot  probably  plays  a 
role — excess  in  walking  (peddlers)  may  aid.  Psychoneurotic  factors 
also  may  play  a  part  in  the  causation  of  these  arterial  cramps  inde- 
pendent of  any  definite  arteriosclerosis.  The  complicated  question 
of  altered  chemism  within  the  vessel  walls  cannot  be  entered  into. 
Hereditarily  inferior  vascular  systems  are  factors. 

Therapy. — Rest,  warm  applications  to  the  parts,  and  high  frequency 
current  application  are  of  value  in  treating  the  attack  in  its  acute 
stage.  Treatment  of  the  condition  rests  upon  the  proper  conception 
of  the  individual  provocative  disorder.  Arteriosclerotic  cases  need 
treatment  for  this;  psychoneurotics  require  psychotherapy.  Of  the 
more  fundamental  therapy  of  the  vegetative  system  which  permits  the 
spasticities  as  well  as  modifies  the  calcium  metabolism  in  the  vascular 
walls  nothing  as  yet  can  be  laid  down. 

Ophthalmic  Migraine. — This  is  also  known  as  sick  headache; 
megrims;  hemicrania;  bilious  headache. 

This  protean  affection  is  difficult  to  define.  It  may  be  a  simple 
or  an  extremely  complex  condition.  Migraine  may,  however,  be 
defined  as  a  periodical  abnormal  state  in  which  the  patient  suffers 
from  a  peculiar  oppressive  pain  in  the  head,  unilateral  or  bilateral, 
localized  or  general,  which  develops  very  gradually  from  heaviness  to 
dulness,  to  pain  that  is  splitting,  and  is  accompanied  or  more  often 
preceded  by  characteristic  visual  signs,  such  as  scotomata,  flying  specks, 
or  partial  blindness.  Chilliness,  depression,  and  sensory  disturbances, 
particularly  in  the  stomach,  and  which  may  lead  to  nausea  or  vomiting, 
are  also  usually  present.  An  attack  may  be  terminated,  after  a  few 
minutes,  by  vomiting,  or  it  may  persist  hours  or  even  days.  After  a 
variable  length  of  time,  usually  following  a  heavy  sleep,  the  patient 
regains  his  previous  condition  of  well-being.  Nearly  everyone  has  an 
attack  or  attacks  of  migraine  during  a  life-time,  hence  its  extended 
description  here. 

1  Frankl-Hochwart,  Deut.  Zeit.  f.  Nervcnheilk,  1913,  vol.  xlvii  and  xlviii. 


OPHTHALMIC  MIGRAINE  105 

History. — A  heritage  of  the  rich  and  the  poor,  the  great  and  the  small 
alike,  it  has  numbered  among  its  sufferers  many  of  the  master  minds  of 
all  times,  and  no  disorder  can  vie  with  it  in  richness  of  description  from 
medical  writers  who  have  been  themselves  subject  to  its  vagaries.^ 
Aretaeus  is  credited  with  having  given  the  first  description  of  migraine. 
Celsus  gave  a  description  which,  while  not  corresponding  in  many 
details  with  what  is  now  understood  to  be  migraine,  is  nevertheless 
very  suggestive.  Caelius  Aurelianus  noted  for  the  first  time  that  the 
Greeks  called  it  hemicrania.  Lepois,  in  the  seventeenth  century, 
gave  his  personal  experiences  through  fourteen  years,  and  called  atten- 
tion to  the  fact  that  the  usual  after-effects  of  vomiting  and  sopor  might 
come  on  without  the  presence  of  the  headache.  Wepfer  in  the  same 
century  seems  to  have  more  clearly  appreciated  the  eye  symptoms. 

Tissot's  description,  1784,  remained  authoritative  up  to  the  appear- 
ance of  Liveing's  monograph,  "On  Megrim,  Sick  Headache,  and 
Some  Allied  Disorders"  (1873),  although  in  the  interim  the  symp- 
tomatology was  becoming  richer  and  the  case  analyses  more  exhaustive. 
Thus,  Vater,  Hennicke,  and  Heberden  made  observations  upon  the 
scotomata.  Plenck,  Parry,  Wollaston  drew  from  personal  experiences 
the  picture  of  half-sided  blindness.  Schonlein  and  Romberg  introduced 
the  neuralgic  theories,  while  Dubois-Reymond,  influenced  by  the  newer 
work  of  Claude  Bernard,  developed  the  hypothesis  of  arterial  spasm 
which  Mollendorf  controverted,  and  postulated  a  sympathetic  paraly- 
sis, both  of  which  views  were  conciliated  by  Jaccoud  and  by  Eulenberg 
(1867),  who  described  angiotonic  and  angioparalytic  conditions. 

Etiology. — The  general  hypothesis  of  a  vasomotor  disturbance  acting 
through  the  vegetative  nervous  system,  seems  to  account  for  most  of 
the  facts,  and  the  general  position  here  taken  is  that  such  disturbance 
may  be  conditioned  by  a  host  of  causes.  The  view  advocated  then 
admits  that  a  certain  amount  of  fact  exists  in  practically  all  of  the 
hypotheses  but  maintains  that  a  one-sided  mode  of  interpretation  is 
inadmissible. 

Abortive  Attacks. — Incomplete  or  abortive  attacks  may  be  said  to  be 
the  rule  rather  than  the  exception  and  attempts  to  classify  the  disorder 
according  to  the  number  of  symptoms  present  offer  no  help  in  the  under- 
standing of  the  complete  picture. 

Mobius  suggests  that  the  parents  of  patients  suffering  from  migraine 
with  scotomata  often  have  suffered  from  migraine  without  scotomata, 
but  he  also  speaks  of  the  reverse  as  happening.  The  extreme  preva- 
lence of  migraine  makes  many  of  the  conceptions  regarding  its  neces- 
sary hereditary  nature  very  dubious,  and  the  extreme  variability  of 
the  individual  attacks  in  the  same  patient  makes  general  hereditary 
features  extremely  improbable.  It  is  by  no  means  infrequent  to 
find  patients  that  show  at  one  time  or  another  almost  every  symptom 
mentioned  in  the  voluminous  literature  of  migraine.    Thus,  one  patient 

1  See  article  by  Jelliffe:  Osier,  Modern  Medicine. 


106  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

under  personal  observation,  had  about  two  attacks  weekly  for  a  year. 
He  then  went  two  years  without  a  single  attack,  and  he  then  had 
several  severe  ones  with  aphasia  and  psychical  symptoms,  interspersed 
with  abortive  attacks,  with  hardly  any  two  alike.  He  was  a  veritable 
museum  of  migraine  attacks  in  the  fifteen  3'^ears  that  he  was  under 
observation. 

Many  families  are  known  in  which  both  parents  have  been  sufferers 
from  chronic  migraine  for  years,  and  yet  none  of  the  children,  now  in 
some  instances  over  forty  years  of  age,  have  ever  had  more  than  one 
or  two  attacks.  The  high  percentage  of  incidence  makes  it  almost 
impossible  to  calculate  an  hereditary  factor.  Again,  it  may  be  borne 
in  mind,  that  as  there  are  many  kinds  of  epilepsies,  so  also  there  are 
undoubtedly  many  migraines.  Some  are  due  to  hereditary  anomalies, 
while  others  have  nothing  to  do  with  anything  of  an  hereditary  char- 
acter. Thus,  one  can  speak  of  migraines  that  are  possibly  hereditary 
and  others  that  are  not. 

The  commonest  abortive  attacks  are  those  that  begin  in  the  classical 
manner,  with  chilliness,  perhaps  with  pinched  face,  and  cold  extrem- 
ities. The  patient  then  has  the  scotomata  and  wretchedness,  depres- 
sion and  apprehension,  and  then  while  waiting  for  the  headache  the 
patient  notices  that  it  does  not  come,  and,  although  he  may  still  have 
heaviness  and  a  sense  of  discomfort,  the  feeling  of  relief  is  sufficient  to 
make  him  feel  well. 

Others  have  added  the  sensation  of  prickling  in  the  fingers,  numbness 
in  the  hand  or  arm,  or  other  sensory  disturbances  without  the  headache. 
In  some  the  entire  attack  will  consist  of  a  disturbed  painful  sense  of 
discomfort,  without  sensory  symptoms,  scotomata,  or  headache, 
but  they  feel  sick  at  the  stomach,  and  have  an  attack  of  what  they 
term  ''biliousness,"  which  clears  up  after  vomiting.  This  feeling  will 
recur  with  sufficient  frequency,  and  at  times  be  combined  with  such 
other  symptoms  of  a  migraine  attack,  in  its  varying  aspects,  as  to 
stamp  the  whole  process  as  a  variant  of  a  true  attack.  Isolated  attacks 
of  vomiting  as  the  sole  expression  of  a  migraine  are  known. 

Attacks  of  scotomata  occur  alone,  without  antecedent  distress, 
and  no  after-attacks  are  noted.  These  are  most  uncommon.  Histor- 
ically it  may  be  noted  that  Parry  and  Airy  had  such  attacks.  It  is 
highly  probable  that  the  majority  of  patients  who  have  had  many 
migraine  attacks  will  have  had  some  of  this  nature.  Attacks  of  scoto- 
mata and  vomiting  occur  without  headache.  In  many  on  the  contrary 
headache  is  the  only  symptom. 

Some  patients  have  attacks  of  hemiparesthesia  with  no  other 
symptoms  of  migraine.  These  generally  occur  at  night,  and  usually 
follow  severe  mental  exertion;  in  one  patient  under  observation  a 
severe  ordeal  in  playing  a  difficult  piece  of  music  will  bring  on  such  an 
attack  without  other  signs.  This  patient's  severe  attacks  are  very 
extreme,  being  associated  with  hemiedema,  hemiparesis,  hemianes- 
thesia, and  marked  hysteromaniacal  outbursts. 


OPHTHALMIC  MIGRAINE  107 

Under  the  heading  of  equivalents,  Liveing  speaks  of  stomach  attacks 
associated  with  some  of  the  vascular  phenomena  of  migraine;  glossal 
spasms  are  also  mentioned  by  him.  Attacks  of  giddiness,  vertigo, 
intestinal  colic,  mental  anxiety  and  depression  which  occur  period- 
ically in  partial  association  with  migraine  symptoms,  are  also  noted 
as  equivalents.  There  is  need  of  further  study  of  these  isolated 
phenomena  associated  with  vasomotor  disturbances. 

Attempts  have  been  made  to  determine  the  relative  frequency  of 
migraine  attacks  with  and  without  the  visual  signs.  These  are  not 
over-reliable,  because  of  the  vast  preponderance  of  abortive  attacks 
over  those  of  the  complete  classical  type. 

Mobius  expresses  the  opinion  that  the  percentage  of  visual  accom- 
paniments of  the  attacks  is  usually  overstated.  His  statistics  show 
130  cases,  with  14  visual  aura.  In  Liveing's  60  patients,  37  suffered 
from  scotomata.  Gowers  says  that  the  cases  are  about  half  and  half, 
with  and  without  eye  signs.  Galezowski  maintains  that  the  visual 
aura  migraines  appear  later  in  life,  thirty  to  fifty  years,  than  ordinary 
migraines. 

It  is  difficult  to  state  an  individual  position,  the  results  of  personal 
inquiries  having  been  so  diverse.  Close  questioning  has  revealed 
the  fact  that  at  some  time  or  other  in  the  course  the  majority  have  had 
visual  symptoms,  and  it  is  not  improbable  that  the  usual  statistics 
are  largely  derived  from  studies  of  too  few  attacks,  i.  e.,  largely  from 
the  severer  attacks  only.  Some  notes  on  individual  histories  are  of 
interest.  Several  patients  have  kept  fairly  accurate  records  of  their 
migraine  attacks  for  several  years.  One  shows  168  attacks  in  a  period 
of  about  ten  years;  of  these,  about  100  were  abortive  attacks,  the  vast 
majority  of  which,  60  per  cent.,  consisted  of  scotomata  alone.  Of  the 
68  remaining  attacks,  about  50  per  cent,  were  ordinary  hemicrania, 
lateral  or  bilateral,  without  scotomata,  the  others  ophthalmic  migraine, 
usually  unilateral  and  with  scotomata.  Not  one  of  the  attacks  was 
ever  accompanied  by  vomiting.  Two  were  associated  with  aphasia, 
fifteen  with  sensory  tactile  associations;  there  were  five  or  six  attacks 
of  hemiparesthesia,  one  in  the  day-time,  the  rest  at  night.  Spasms 
of  the  orbicularis  were  a  common  accompaniment.  Every  attack  suffi- 
ciently severe  to  require  an  analgesic  was  promptly  relieved  by  from 
5  to  10  grains  of  either  antipyrin,  acetanilid,  or  phenacetin. 

Classical  Migraine. — Early  Symptoms.— These  may  be  termed  pre- 
cursors of  a  full  attack  of  migraine,  or  they  may  constitute  the  symp- 
toms of  an  abortive  attack.  The  most  striking  are  a  sense  of  heaviness, 
with  yawning,  chilliness,  dizziness,  or  depression,  motor  twitching, 
even  sharp  spasmodic  closure  of  the  eyelids,  sensory  phenomena, 
chiefly  paresthesise,  occasionally  anesthesia,  and  affections  of  the 
eyes  or  other  sensory  organs,  ringing  in  the  ears,  blowing,  whistling, 
modifications  of  taste,  of  smell,  of  touch,  etc.  There  may  be  failure 
of  appetite,  constipation,  diarrhea,  vascular  instability,  hot  flashes 
chasing  here  and  there  over  the  body,  throbbing  in  the  carotids,  etc. 


108  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

The  temporal  arteries  are  often  smaller,  the  saliva  diminished,  and 
the  pupils  narrowed. 

The  premonitory  signs  which  show  a  great  deal  of  variability  in 
different  individuals,  and  also  in  different  attacks  in  the  same  indi- 
vidual, may  be  felt  several  minutes  before  the  attack,  in  some  rare 
instances  even  days.  This  is  frequently  the  case  in  women  in  whom 
the  onset  of  the  menstrual  function  seems  to  bear  some  relation  to  the 
attack.  The  ordinary  depression  felt  at  this  time  is  a  thing  apart 
from  this  special  type  of  depression  that  pervades  them.  At  times 
such  attacks  of  depression  and  anxiety,  combined  with  a  sense  of 
chilliness  and  dizziness,  will  constitute  the  entire  picture  of  the  abor- 
tive attack.  Many  attacks  come  apparently  without  the  slightest 
warning. 

Many  patients  having  attacks  at  night  find  themselves  heav}^ 
and  tired,  with  sore  spots  on  the  scalp  in  the  morning.  Mobius 
relates  a  case  in  which  the  patient  dreamed  of  having  swallowed 
a  rabbit,  which  ate  its  way  out  through  the  stomach  wall.  After 
this  unpleasant  dream  the  patient  had  a  severe  migraine  on 
awakening. 

In  all  probability  premonitory  symptoms  of  some  type  are  invariably 
present;  when  thought  to  be  absent  it  is  because  the  patient  has 
overlooked  them,  either  by  reason  of  their  mild  character,  or  because 
of  naturally  poor  powers  of  observation.  Many  patients,  who  have 
had  headaches  for  years,  have  never  noticed  their  one-sided  localization, 
or  the  well  known  fortification  spectra,  until  their  attention  has  been 
directed  specifically  to  them.  Many  patient's  will  deny  ever  having 
had  zig-zags  of  light,  etc.,  until  shown  Airy's  pictures,  when  they 
remember  having  seen  such  phenomena.  It  is  because  of  such  poor 
observation  that  many  cases  of  true  migraine  are  overlooked,  which 
fact  lends  further  support  to  the  belief  that  this  disorder  is  very  much 
more  prevalent  than  is  usually  supposed. 

Sensory  Symptoms. — In  the  more  classical  attacks  the  patient  has 
preliminary  sensory  symptoms.  These  are  spoken  of  by  Mobius  in 
the  sense  of  an  aura.  If  the  term  aura  be  used  as,  for  instance,  the 
term  "fever"  is  used,  there  can  be  no  objection,  but  if  by  an  aura  is 
meant  a  restricted  phenomenon  essentially  related  to  an  epileptic 
aura,  the  term  should  be  eliminated. 

A  sense  of  coldness  and  chilliness  is  one  of  the  commonest  sensations. 
This  is  usually  general,  and  is  associated  with  a  pale  countenance, 
gooseflesh,  perhaps  clammy  hands,  and  a  sense  of  misery.  Cases 
are  known,  and  are  by  no  means  uncommon,  in  which  the  chilliness 
has  been  one-sided,  and  is  accompanied  by  other  phenomena  involving 
one-half  of  the  body,  including  the  face,  of  the  same  side.  Yawning  is 
a  common  early  sign. 

Unilateral  paresthesia  is  not  an  uncommon  early  sign.  Many 
patients  note  a  tingling  or  numbness  in  the  fingers  of  one  hand;  this 
may  spread  up  the  arm,  and  in  rare  instances  general  unilateral  pares- 


PLATE    IV 


From  Dr.  Hubert  Airy's  Paper,  On  a  Distinct  Form  of  Transient 
Hemiopsia,  Philosophical  Transactions,  1870,  p.  247. 

Figs.  1  to  4.— Early  stages  of  Fortification  Spectra  as  seen  in  dark.     0=  sight  point. 

Figs.  5  to  8. — Similar  series,  beginning  lower. 

Fig.  9 — Fully  developed.     i5^  =  secondary  attack  within. 


OPHTHALMIC  MIGRAINE  109 

thesia  of  a  very  uncomfortable  nature  may  be  present.  In  some 
instances  such  unilateral  paresthesise  have  constituted  the  sole  symp- 
tom of  an  attack,  save  for  the  heaviness  and  usual  discomfort.  Occur- 
ring at  night,  such  attacks  are  often  extremely  wearing,  keeping  the 
patient  awake.  Photophobia,  flow  of  tears,  strange  sounds,  tinnitus, 
peculiar  odors,  queer  peppery  or  flat  tastes,  may  be  noted. 

Anesthesia  is  less  often  observed,  largely  because  of  the  negative 
character  of  the  symptom.  When  involving  the  face  or  mouth  it  is 
complained  of.  Anesthesia  frequently  follows  the  tingling  of  the  early 
paresthetic  disturbances.  Franz^  has  shown  that  there  is  a  very 
evident  decrease  in  the  pain  threshold,  especially  after  the  headache 
has  set  in. 

The  us2ial  phenomena  are  the  most  striking,  and  hence  held  to  be 
of  the  most  frequent  occurrence.  The  ease  of  observation  in  part 
accounts  for  the  usually  accepted  opinion  that  they  are  the  commonest 
of  the  early  symptoms.  Very  few  individuals  have  been  subjected  to  a 
careful  sensory  examination.  If  more  were  investigated,  it  is  probable 
that  other  slight  sensory  signs  would  be  found  to  be  equally  prevalent 
and  as  evanescent.  The  visual  signs  have  been  described  by  many 
writers,  and  many  illustrations  have  been  made  showing  their  chief 
characteristics.  The  extreme  uniformity  of  their  general  character 
is  striking,  as  well  as  the  variations  of  the  same  pattern. 

As  a  rule,  the  patient  notices  a  slight  blurring  of  his  vision  if  reading, 
or  a  slight  flicker  of  light  located  in  one  eye,  to  one  side  of  the  center. 
Closer  observation  reveals  either  a  slight  cloudy  spot,  which  seems 
to  follow  the  eye  in  reading,  cutting  out  the  after-images,  a  letter 
or  so  from  the  center  of  clear  vision.  The  slight  subjective  sense 
of  difficulty  in  reading  may  precede  the  discovery  of  a  scintillating 
spot  which  becomes  visible  on  closing  the  eyes.  Little  by  little  this 
spot  spreads  out,  usually  in  a  crescent-like  fashion.  General  statistics 
are  thus  far  unavailable,  but  a  special  study  has  shown  that  the 
majority  of  these  scotomata  have  begun  in  the  left  eye,  are  situated 
to  the  left  of  the  middle  line,  with  the  convexity  of  the  crescentic 
border  to  the  left.  As  the  crescent  gradually  grows  larger,  the  difficulty 
in  seeing  clearly  becomes  more  marked,  especially  on  the  periphery 
of  the  visual  field.  For  most,  the  scotomata  is  in  constant  motion, 
flashing  in  its  spectral  zig-zag  fashion,  thus  causing  the  classical 
name  "  fortification  spectrum"  from  the  play  of  colors,  and  the  fortress- 
like "ins  and  outs"  of  the  outline. 

After  a  variable  time,  from  five  to  twenty  minutes,  the  scotomata 
gradually  subsides,  or  suddenly  disappears,  to  be  followed  by  the 
headache.  Not  infrequently  the  headache  never  comes,  and  the 
preliminary  sensory  phenomena  of  chilliness,  heaviness,  and  scotomata 
constitute  an  abortive  attack.  A  description  of  the  scotomata  of 
migraine  might  fill  a  volume.  The  classic  of  Liveing  reproduces  the 
excellent  illustration  of  Airy's,  which  is  here  reproduced. 

'  Amer.  Jour,  of  Physiology,  1905, 


110 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


Occasionally  the  right  half  of  the  field  is  involved.     Sometimes  it  is 
the  upper  half,  one  of  Mobius'  patients  saying  that  everybody  seemed 

THE    DEVELOPMENT    OF    SCOTOMATA   IN   MIGRAINE. 


have  not  studied  immigrati 
:ers  into  which  immigrati 
esire  for  larger  opportuniti 
always  bspj^  the  prime  fore 
.  This  5fwet-J|<nas  proved 
ie  any  re  J^tion  of  immigr 
m  for  the  selection  of  imn; 
'  desirable  it  is  to  apprecia 
:olonization  and  to  examij 
:en  place  in  the  causes  ai 


There  has  been  no  sue 
the  United  States  as  thj 
longingfor  liberty  and  fi 
for  lai^^mmigration  at 
settleniB^  gL  America;  i 
Island  ^fjfce  present  tim 
and  th^^ntiersman  of 
that  a  very  large  numbei 
lured  by  purely  economic 
rights,  many  immigrants 


Fig.  38. — Stage  of  blurring  three  minutes.  Fig.  39. — First  outlines  of  scintillations. 


111.1,0.  j^iBuuBSion  ot  immigration 
i  statements  that  our  forefathers 
)f  ir^migrants, 
y,' new' genera ' 
e  back  the  I 
efly  to  imm: 
'tor  the  toi 
estate  the  d( 


questions  is- usually  introduCec 
were  immigrants  and  the  ct 
following  theCte&nial  period 
their  kinsia^^that  immigran 
the  West  a^^at  we  areindel 
indusMan^S  conyjercial  gre 
ments  ar^^and  i^s  difficul 
owes  to  j^Sltants.  It  is,  h( 
of  presen#^]^tEttjgration  as  sin 
inspired  by  iMfiise  longing  fo 
find,  it  even  at  the  cost  of  da 
part  of  the  civilization  of  the 
period  and  in  the  years  foUowi 


a  very  serioi 
continuation 
aal  liberty  an 
d  privation,  ^ 

orld  to  the  New  iiTtl 
Revolutionary  War 


Fig.  40. — Five  to  ten  minutes  growing  scotoma.  Fig.  41. — Ten  to  fifteen  minutes. 


of  arrivals  at  Ellis 
.    The  addition 
^le  racial  or  eeoa 

those  wto  had 
ship  as  well  as 
ig  the  next  tWj 

of  immigran 
in  Ireland  in 
t  illustratio 
ms  in  Europff 
America.    It^ 
'eloped  by  im 
iefinite  relatio 
the  number  of  ^ 
.n  Europe.     I^e 
on  on  account  pfj 
'Dsiderable  ext.e  . 
It  it  was  influeticed 


single  mont 
s-000  alien! 
Nearly 
_jfore  and  many  ^ 
"tho^e  with  whom  tl: 
•ars  there  was  a  stes 
.  the  latter  part  of 
iddenly  caused  a  larg 
important  new  soci^ 
.  definite  relation  to  tn 
leny  years  later  than  ai 
.'Stion— that  economic  o 
)HHe  volume  of  immigra 
fn  this  country  who  r 
;o55(fe^rom  Ireland  wai 
re  pf  the  potato  c, 
Jhropic  indiv: 
tooT'bY\ne"^Hie  motiv 


Fig.  42. — Fifteen  to  twenty  minutes. 


?^ 


out,  the  English,  Di 
^     practically  all  arrivals  i 
■oluntionary  War  were  clos 
one  iGermanic  race  in  tl 
ey  colonized  the  Atlantic 
form  the  thin  line  of  civilizat 
of  the  same  race.    The  "imn: 
r^many  years  after  the  Reva^ 
'Jjfan  those  who  had  preceded  the^ 
ive  considered  as  tibat  of  contin 
ivilWar.  During  that  period  al^ 
ppiik  It  is  not  possible  to  learn  the  I 
this  country  before  1820  for  in  t 
to  record  their  number  and  to  ai 
know,  however,  that  the  popi 
Uy  increased  by  immigratic 
oktionary  War.    During 


7as  not 
ollowi 


Fig.  43. — Just  before  disappearing, 
twenty  to  thirty  min^ites,  and  begin- 
ning of  headache. 


headless;  occasionally,  it  is  the  lower.     In  rare  instances  the  patients 
complain  of  total  blindness,  i.  e.,  central  scotomata.     Berbez  reports 


OPHTHALMIC  MIGRAINE  111 

an  interesting  case  of  a  ring-like  scotomata — the  patient,  on  looking 
at  his  watch,  could  see  only  the  central  pin  where  the  hands  were 
united;  the  figures  on  the  dial  were  all  obscured  by  the  scintillating 
scotoma.  These  scotomata  are  usually  bilateral  phenomena.  They 
may  begin  in  one  eye  before  appearing  in  the  other,  and  be  some- 
what different  in  the  two  eyes,  and  may  disappear  in  one  eye  sooner 
than  the  other.     Scotomata  limited  to  one  eye  are  probably  rare. 

The  relationship  of  these  scotomata  to  psychical  symbolizations 
has  not  yet  been  investigated.  In  the  few  cases  thus  far  analyzed, 
left-sided  symptoms  are  apt  to  symbolize  the  love  conflicts,  right- 
handed  ones,  the  nutritive.  Pains  in  the  back  of  the  head  are  frequently 
associated  with  hate  complexes,  as  are  also  jaw  pains. 

The  retinal  occurrences  during  the  time  of  these  scotomata  are 
uncertain.  Blanching  of  the  papillae  has  been  observed  by  some 
(Galezowski) ;  pulsation  of  the  retinal  arteries,  with  dilatation  by 
others.  Personal  experience  has  shown  similar  dilatation  in  a  few 
cases,  but,  as  a  rule,  a  normal  fundus  is  found.  The  picture  seen 
will  depend  upon  the  stage  of  the  attack  and  its  severity. 

Pupillary  dilatation  occurs  late.  Slight  irregularity  of  the  pupils 
during  a  severe  attack  of  an  ophthalmic  migraine,  dilatation  being 
usual  on  the  affected  side,  is  not  unusual.  Bilateral  pupillary  con- 
traction is  the  rule  in  the  headache  stage. 

During  the  onset  of  the  fortification  spectra  it  not  infrequently 
happens  that  mild  motor  phenomena  occur  in  the  eyelid  of  the  side 
to  be  affected.  The  eyelid  droops  a  particle,  and  Gowers  and  others 
report  double  vision,  interpretable  as  a  sign  of  paresis  in  an  ocular 
muscle. 

Motor  Disturbances. — Speech. — ^This  may  be  considered  as  both  a 
motor  and  sensory  phenomenon,  for  the  most  frequent  type  of  change 
is  a  transitory  sensory  aphasia.  Anarthrias  are  known,  especially  in 
the  ophthalmoplegic  variety,  but  for  ophthalmic  migraine  the  type 
of  aphasia  found  is  very  characteristic.  As  described  by  Charcot, 
it  is  an  intermittent,  halting  aphasia.  At  one  moment  the  patient  can 
get  the  right  word,  at  the  next  he  cannot.  He  stumbles  on  a  word; 
uses  madame  for  monsieur,  etc.  In  Liveing's  cases,  15  out  of  20  had 
speech  disturbances;  one  on  hearing  clock  bells  was  unable  to  inquire 
what  they  were.  Fere  cites  the  case  of  a  coachman  who  forgot  where 
he  was  going  to  drive  his  passengers;  Berbez  a  like  case  in  which  a 
pedestrian  lost  his  way,  as  he  could  not  read  the  street  signs  under- 
standingly.  Gowers  speaks  of  a  case  of  word  deafness.  Cases  of 
agraphia  are  also  known.  Mobius  reports  a  case  with  typical  scintillat- 
ing scotomata  at  one  time  on  the  right  side,  at  another  on  the  left. 
When  the  patient  suffered  from  a  right-sided  scotoma  he  had  sensory 
aphasic  signs,  but  they  were  not  present  when  the  scotoma  was  on 
the  left  side. 

Other  observers  have  noted  the  same  phenomena,  while  contra- 
dictory   observations    are    also    recorded.     The    speech   disturbance 


112  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

sometimes  resembles  a  paraphasia,  the  patient  using  a  jumble  of 
words.  In  a  personal  case  the  patient  could  not  sing  a  well-known 
tune  correctly,  his  sense  of  musical  values  having  been  interfered  with. 

The  onset  of  the  aphasic  disturbance  may  vary  greatly.  It  is 
usually  temporary,  persisting  at  times  for  only  a  few  minutes,  again 
persisting  a  few  hours.  It  frequently  antedates  the  headache,  or  is 
coincident  with  it.  In  a  case  reported  by  Meige  the  aphasia  persisted 
as  long  as  the  headache,  and  disappeared,  as  a  rule,  when  that  dis- 
appeared. The  patient  showed  a  loss  of  ability  to  say  certain  words 
and  a  tendency  to  the  employment  of  incorrect  words.  There  was  no 
anarthria. 

Cerebellar  Symptoms.- — Oppenheim  has  called  attention  to  a  cere- 
bellar hemicrania  in  a  patient  in  whom  every  attack  of  migraine  was 
accompanied  by  typical  cerebellar  symptoms.  The  patient  was 
uncertain  in  his  gait,  walked  like  a  drunken  man,  was  dizzy,  and 
had  the  sensation  that  his  body,  or  individual  parts  of  it  were  doubled. 
The  sense  of  equilibrium  was  disturbed  in  each  attack.  Dizziness 
and  loss  of  the  sense  of  equilibrium  are  not  infrequent  but  such  a 
complete  syndrome  has  been  described  only  by  Oppenheim. 

Paralytic  Phenomena. — Attention  has  already  been  called  to  the 
rare  occurrence  of  hemiparesis,  which  may  even  involve  the  facial 
muscles.  Up  to  the  present  time  no  instances  of  crossed  hemiplegic 
types  have  been  found  in  the  literature.  This  is  of  interest  in  connection 
with  the  hypothesis  of  the  bulbar  origin  of  migraine,  especially  of  the 
ophthalmoplegic  variety. 

Headache. — This  is  the  most  common  feature  and  exhibits  a 
great  amount  of  variability  as  to  location,  quality,  intensity,  and 
duration.  In  the  more  classical  attacks  the  headache  begins  on 
the  average  about  fifteen  to  thirty  minutes  after  the  appearance  of 
the  scotomata  or  other  sensory  phenomena.  It  frequently  begins 
on  one  side,  and  may  remain  so  or  become  bilateral.  As  a  rule, 
it  is  frontal,  or  occupies  the  vertex,  but  may  involve  the  temporal 
regions,  the  occiput,  sometimes  as  low  down  as  the  neck.  Gowers' 
experience  points  to  the  parietal  region  as  being  oftenest  affected, 
and  usually  over  a  small  area.  Henschen,  in  123  patients,  shows 
the  pain  to  have  been  located  110  times  in  the  forehead,  100  times  in 
the  parietal  region,  and  54  times  in  the  occiput.  There  is  usually  pain 
over  the  eyes,  and  the  eyeballs  are  usually  painful  to  pressure.  In  a 
few  instances  pressure  over  the  malar  bones  is  painful,  and  occasionally 
there  is  a  well-marked  jaw  ache. 

Statistics  of  the  percentage  of  different  locations  are  uncertain  since 
the  individual  will  have  all  the  different  varieties.  Thus,  in  a  case 
already  cited,  in  which  the  abortive  attacks  were  so  frequent,  the 
headaches  comparatively  rare,  the  strictly  unilateral  headaches  were 
only  5  per  cent,  of  the  entire  number.  In  others  the  hemicranic 
type  runs  much  higher.  In  Henschen's  records  of  123  cases,  56  had 
one-sided   attacks,   in   67  both  sides  were  involved.     In  Liveing's 


OPHTHALMIC  MIGRAINE  113 

61  patients,  17  had  one-sided  attacks,  in  7  the  attacks  were  variable, 
while  in  34  both  sides  were  involved.  Mobius  and  others  note  that 
the  headache  often  appears  on  the  side  opposite  to  that  affected  by 
the  sensory  aura.  Personal  studies  do  not  confirm  Mobius'  statement. 
It  does  seem,  however,  as  first  noted  by  Liveing,  that  one-sided 
sensory  symptoms  are  oftener  accompanied  by  one-sided  than  by 
bilateral  headaches.  With  bilateral  sensory  phenomena,  scotomata, 
etc.,  bilateral  pains  are  the  commonest. 

In  many  attacks  the  pains  are  limited  to  the  eyes,  the  feeling  of 
soreness  of  the  eyeballs  being  so  very  marked  that  it  is  painful  to  move 
them.  Pain  in  the  neck  may  also  cause  the  desire  to  hold  the  neck 
rigid. 

The  character  of  the  pain  defies  analysis,  since  descriptive  phrases  are 
used  in  such  various  ways  by  different  observers.  In  some  attacks,  the 
head  simply  feels  slightly  sore,  or  heavy,  or  dull,  or  thick;  "like  a 
block  of  wood,"  is  a  frequent  expression.  "Filled  with  sawdust," 
one  patient  says.  Again,  the  pain  is  agonizing,  impossible  to  describe. 
Some  patients  shriek  with  the  pain,  become  hysterical,  and  roll  about 
the  floor,  grasping  the  head  between  the  hands,  wishing  to  beat  their 
brains  out.  Between  these  extremes  numberless  variants  are  found 
among  different  individuals,  and  in  different  attacks  in  the  same 
individual.  Nearly  all  patients  will  say  that  the  severe  pains  are 
throbbing  or  thumping,  usually  indicating  great  pressure  from  within 
or  without;  as  Mobius  has  said,  "some  patients  think  the  head  will 
burst,  others  that  it  is  being  squeezed  in  a  vise."  Descriptions  of 
bursting  are  more  common.  The  pain  is  an  all-pervading  one,  grad- 
ually mounting  to  a  maximum,  then  running  along  continuously 
without  any  let  up,  with,  at  all  times,  sudden  accessions,  especially 
on  movement,  if  one  leans  over,  or  is  forced  to  sudden  exertion.  In 
but  the  rarest  instances  is  it  described  as  lancinating  in  quality.  It  is 
the  type  of  pain  apparently  seen  in  cerebral  tumor,  in  acute  hydro- 
cephalus, in  cerebrospinal  meningitis,  and  is  allied  to  the  pain  of 
opium  poisoning,  or  of  sea-sickness;  all  pointing  in  the  direction  of  a 
modification  of  intracerebral  pressure,  at  times  an  increase,  or  it  may 
be  a  decrease,  either  of  which  may  cause  severe  pain.  Occasionally 
the  phenomenon  of  a  bilateral  headache  with  marked  predominance 
of  one-sided  pain  will  be  observed. 

The  severity  of  the  pain  may  be  conditioned  by  a  number  of  factors. 
Movement  uniformly  increases  it.  Bending  over  becomes  impossible. 
The  first  movement  on  lying  down  is  usually  accompanied  by  a  sudden 
rise  in  severity,  but  this  gradually  subsides.  The  taking  of  alcohol 
usually  increases  the  severity  of  the  pain,  as  does  also  the  use  of  tobacco. 
Eating,  if  possible,  may  help  somewhat,  but  usually  augments  the 
pain,  and  is  avoided.  Strong  sensory  impressions  invariably  increase 
the  pain.  Noises  of  various  kinds  often  aggravate  the  pain  tremen- 
dously and  cause  certain  patients  marked  distress.  The  "Fourth  of 
July"  invariably  drives  many    migrainous    patients  to  some  quiet 


114  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

spot  in  the  country,  free  from  crackers  and  bombs.  Mobius  notes 
that  the  rage  of  migrainous  parents  directed  toward  their  noisy  children 
often  resembles  a  pathological  hatred.  Strong  light  is  invariably 
avoided,  because  of  its  tendency  to  increase  the  pain.  The  movements 
of  the  eyeball  and  attempts  at  visual  accommodation  cause  an  increase 
in  the  pain. 

Psychical  effort  is  often  impossible;  in  milder  attacks  the  awakening 
of  a  strong  mental  stimulus  may  make  one  forget  the  pain.  Mobius 
says  that  his  attacks,  usually  light  ones,  are  frequently  forgotten  during 
an  interesting  visit  to  the  Policlinik,  to  be  once  more  prominent 
afterward.  One  of  us  has  frequently  begun  a  lecture  with  a  severe 
migraine  to  find  it  almost  forgotten  until  the  close,  when  it  reappears, 
usually  with  renewed  vigor. 

The  movements  of  straining  at  stool,  and  vomiting,  coughing,  etc., 
invariably  cause  a  rapid  and  sharp  rise  in  the  severity  of  the  pain. 
Sensory  stimuli  may  have  an  unpleasant  effect  on  the  psyche.  Thus, 
certain  odors  cause  distress;  the  smell  of  cooking  acts  much  as  it  does 
on  shipboard;  it  accelerates  vomiting.  Certain  skin  phenomena,  such 
as  sore  spots,  are  frequent  after  the  headaches. 

In  certain  personal  experiments  with  drugs  the  following  have 
invariably  increased  the  headache  within  a  few  minutes:  A  few 
whiffs  of  chloroform  or  of  ether,  adrenalin  by  mouth,  digitalis,  stro- 
phantin,  and  ergot.  Drugs  that  raise  the  blood-pressure,  in  general, 
increase  the  pain  when  taken,  especially  at  the  beginning  of  the  head- 
ache. The  headache  may  clear  away  very  suddenly  after  an  attack 
of  vomiting,  or  it  may  pass  without  vomiting;  in  some  it  fades  away 
gradually.  It  may  last  a  few  minutes,  a  few  hours,  or  a  few  days. 
Some  cases  of  what  Mobius  chooses  to  call  status  hemicranicus  are 
recorded. 

Vasomotor  Disturbances. — Practically  all  attacks  of  migraine  are 
accompanied  by  visible  vasomotor  disturbances.  In  most  cases 
vasoconstrictor  phenomena  (coldness,  paleness,  gooseflesh,  etc.) 
precede,  to  be  followed  later  by  vasodilator  changes.  Thomas  and 
Cornu  both  point  this  out  as  a  result  of  their  experiences.  Thomas 
contributes  a  statistical  study  of  107  cases  in  support  of  the  early 
pallor,  small  pulse  and  coldness,  which  pass  over  to  the  phenomena 
of  warm,  red,  flushed  face  and  skin,  and  full  pulse.  The  period  of 
initial  constriction  may  be  unnoticed  by  reason  of  its  transitory 
character.  In  some  instances  this  initial  vasoconstriction  may  be 
very  marked  and  give  rise  to  the  phenomena  of  localized  cyanosis, 
even  advancing  to  the  picture  of  the  constriction  phase  of  the  Raynaud 
disease  type. 

In  the  same  manner  the  secondary  vasomotor  dilatation  may 
pass  the  bounds  ordinarily  observed  and  lead  to  localized  edema, 
to  the  erythromelalgic  type,  or,  exceptionally,  to  hemorrhagic 
phenomena  in  the  conjunctiva,  ocular  tissues,  or  even  in  the  walls 
of  the  stomach. 


OPHTHALMIC  MIGRAINE  115 

Secretions. — Alteration  in  secretory  functions  are  frequently  observed 
early  or  late  in  the  attacks,  Reference  has  been  made  to  the  excess 
of  secretion  of  tears  as  a  frequent  precursor.  Vomiting  of  frothy 
mucus,  serous  diarrhea,  increase  of  sweat,  coryza  (Calmeil),  or  inces- 
sant salivation  (Liveing,  Tissot)  are  common  phenomena. 

The  changes  in  urinary  secretion  have  attracted  careful  attention. 
The  early  vasoconstriction  of  the  periphery,  coldness,  lack  of  secretion, 
of  perspiration,  etc.,  account  in  a  purely  mechanical  way  for  the  increase 
of  urinary  secretion  in  the  early  stages.  Metabolic  studies  show  no 
fundamental  disturbances.  Bioglio  was  unable  to  show  constant 
changes.  Although  it  is  not  possible  to  exclude  metabolic  disturbance 
as  causing  changes  in  vegetative  control,  probably  it  is  more  true 
that  psychical  influences  cause  the  metabolic  disturbance. 

Trophic  Disturbances. — These  have  been  reported  by  several 
observers.  Cornu  says  that  nearly  all  of  his  cases  of  migraine  show 
facial  asymmetry  and  facial  atrophy  is  recorded.  These  instances 
are  nearly  always  coincidences  and  are  not  necessarily  attributes 
of  the  migraine.  A  facial  atrophy  which  can  be  interpreted  only  on 
the  basis  of  a  migrainous  disturbance  of  the  vasomotor  apparatus  is 
very  problematical,  and  certainly  Cornu's  results  are  not  confirmed 
by  others.  Loss  of  weight  in  the  severe  rapidly  recurrent  cases  is  due 
to  disturbance  in  general  nutrition  due  to  gastric,  rather  than  to 
other  causes.  Herpes  is  a  not  infrequent  accompaniment  of  some 
cases,  but  the  recognition  of  its  infectious  nature  has  successfully 
disposed  of  its  essential  relationship  to  migraine. 

Psychical  Disturbances. — These  have  been  noted  by  many  observers, 
Liveing  being  one  of  the  first  to  point  out  the  relationship  of  disturbed 
psychical  states  to  the  attacks  of  migraine. 

In  the  vast  majority  of  "migraine  attacks  there  are  no  mental  changes, 
either  before,  during,  or  after  the  attacks.  Mild  depression,  hope- 
lessness, despondency  with  clear  consciousness,  are  frequent  mental 
states.  With  very  severe  pains  Mobius  admits  clouding  of  conscious- 
ness, and  is  not  sure  that  severe  stuporous  states  are  not  due  to  pain 
as  well.  Mingazzini,  on  the  other  hand,  believes  there  is  justification 
for  erecting  a  special  group,  which  he  has  termed  the  hemicranic 
dysphrenias,  and  distinguishes  a  transitory  and  a  more  permanent 
variety.  Recent  observers  are  practically  in  accord,  in  showing  that 
severe  mental  disturbances  varying  in  character  and  intensity  may 
be  part  of  a  migraine  attack. 

Guidi  has  amplified  these  observations  by  reporting  the  history  of 
a  number  of  cases  in  which  the  patients  suffered  during  the  day 
before  the  onset,  in  a  much  more  decided  manner  than  by  feelings  of 
anxiety  or  depression  as  described  by  Liveing.  Thus  Guidi  calls 
attention  to  grave  alterations  in  the  psychical  state  of  a  number  of  his 
patients.  In  one  the  entire  character  of  the  personality  would  change 
preceding  the  attack.  A  patient  who  had  always  been  calm,  reserved, 
quiet,  and  modest,  suddenly  became  much  agitated,  was  forward, 


116  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

noisy,  and  loquacious,  and  told  salacious  stories,  which  was  far  from 
his  usual  behavior.  While  in  health  a  spare  eater,  preceding  an  attack 
he  suddenly  became  very  hungry,  and  hankered  especially  for  starchy 
foods.  During  the  attack  the  patient  had  glycosuria,  which  dis- 
appeared later. 

With  the  onset  of  pain  the  picture  is  less  clear,  yet  there  is  little 
doubt  that  many  patients  suffer  from  profound  psychical  disturb- 
ances, which  arise  independently  of  the  pain.  One  such  case,  under 
personal  observation,  would  be  interpreted  by  Mobius,  and  rightly 
so,  as  one  in  which  the  pain  is  the  first  link  in  a  hysterical  reac- 
tion. But  there  are  other  cases  which  do  not  belong  to  this  group. 
Mingazzini's  hemicranic  dysphrenias  may  be  cited  as  examples, 
in  part,  at  least.  In  others  severe  disturbances  have  occurred,  such 
as  states  of  anxiety,  rising  to  actual  anguish  (Charcot);  phobias  of 
inability  to  perform  acts  (Cornu-Charcot) ;  terror  (Liveing,  Fere, 
Kraft-Ebing) ;  hallucinations  of  sight  (phosphenes,  colored  lights, 
animals)  and  hearing  with  mental  confusion  (Forli,  Mingazzini); 
maniacal  excitement  (Mingazzini,  Jelliffe)  and  stupor;  unconscious- 
ness (many  authors). 

Liveing  reports  that  25  per  cent,  of  his  cases  showed  psychical 
symptoms.  The  Italian  observers  record  fewer,  but  it  appears  that 
at  least  from  10  to  15  per  cent,  of  the  cases  of  grave  hemicrania 
show  some  distinct  mental  disturbance  in  some  one  or  more  of  their 
attacks  which  is  more  significant  than  the  usual  depression  which  is 
so  universal. 

Symptomatic  Migraines. — The  occurrence  of  migraine-like  attacks 
accompanying,  or  due  to,  definite  disease  conditions,  notably  organic 
disease  of  the  brain,  is  well  known.  The  association  of  migraine  with 
gout  and  malarial  affections  has  been  noted.  So  far  as  gout  as  an 
etiological  factor  is  concerned,  Mobius  is  inclined  to  see  nothing 
more  than  a  coincidence;  while,  as  for  malaria,  he  holds  it  to  cause  an 
orbital  neuralgia,  not  a  migraine.  As  for  the  latter,  it  seems  clear 
that  the  well-known  effects  of  malarial  infection  on  bloodvessel  tonus 
are  entirely  sufficient  to  cause  a  typical  migraine  attack.  It  is  known 
that  attacks  of  migraine  may  be  very  frequent  during  the  continuance 
of  a  malarial  infection.  Such  may  disappear  for  months  after  quinine 
therapy,  and  then  reappear  at  the  time  of  a  later  malarial  infection. 

Migraine-like  attacks  are  not  infrequent  in  cerebral  tumor;  they 
may  appear  periodically,  as  in  cases  fully  reported  by  Abercrombie 
and  Mobius,  or  they  may  be  continuous  and  distinguishable  with 
great  difficulty  from  the  pain  of  tumor,  as  in  cases  reported  by  Wer- 
nicke, who  has  said  that  such  attacks  may  be  quite  readily  confused 
with  those  more  typical  of  tumor.  In  tumors,  however,  vomiting 
brings  little  or  no  relief;  quiet  gives  less  relief,  and  the  fluctuation  in  the 
intensity  of  the  pain  is  less  prominent.  A  primary  onset  of  migraine- 
like attacks  in  adult  life  should  always  awaken  the  suspicion  of  an 
organic  brain  lesion. 


OPHTHALMIC  MIGRAINE  117 

Oppenheim  has  called  particular  attention  to  the  occurrence  of 
migraine-like  attacks  at  the  onset  of  tabes;  Mobius  is  inclined  to  think 
it  a  rare  combination,  and  regards  it  either  as  a  pure  coincidence 
or  a  migraine-like  neuralgia.  In  general  paresis,  migraine-like  attacks 
may  be  an  initial  symptom.  Migraine  attacks  are  not  infrequent 
throughout  the  early  stages  of  the  disease,  but  the  anatomical  correla- 
tions are  still  hypothetical. 

Diagnosis. — The  difficulties  appear  in  the  consideration  of  ordinary 
headaches  and  in  neurasthenic  headaches;  in  distinguishing  between 
the  scotomata  of  migraine  and  other  scotomata;  the  paresthesia  of 
migraine  and  other  paresthesias;  the  aphasia,  the  vomiting,  etc., 
as  seen  in  migraine,  and  the  same  as  due  to  other  causes.  In  most 
individuals  abortive  and  incomplete  attacks  are  the  rule,  and  it  is 
often  extremely  difficult  to  determine  their  precise  significance. 

Mobius  has  suggested  that  the  problem  is  not  only  whether  the 
case  is  one  of  migraine  or  not,  but  whether  it  is  migraine  alone,'  and 
not  something  additional.  This  author's  contention  that  migraine  is 
hereditary  and  begins  in  youth,  would  seem  to  make  it  a  simple  matter, 
but  clinical  experience  shows  that  real  migraines  do  appear  in  later 
years,  apart  from  other  affections,  and  as  for  the  hereditary  factor, 
the  extreme  prevalence  of  the  affection  makes  it  hard  to  accurately 
weigh  this  factor.  The  periodic  recurrence  is  a  difficult  criterion. 
There  is  usually  no  difficulty  in  diagnosing  the  classical  attacks  from 
simple  headache,  but  at  times  such  differentiation  is  impossible.  Many 
chronic  sufferers  from  migraine  know  well  their  real  attacks,  are  able 
to  distinguish  abortive  attacks,  and  also  have  headaches  of  an  entirely 
different  nature.  The  simplest  test  in  separating  abortive  migraines 
from  simple  headaches  is  the  occurrence  of  sensory  phenomena,  other 
than  pain,  which  have  their  main  origin  in  vasomotor  disturbances. 
It  is  on  this  account  we  would  ally  the  severe  headaches  following 
the  use  of  alcohol,  ether,  chloroform,  opium,  or  related  drugs  to  the 
migraines  rather  than  to  simple  headaches.  The  headaches  of  neu- 
rasthenia, anemia,  syphilis,  lead  poisoning,  nasal  sinus  involvement, 
supra-orbital  neuralgia,  nephritis,  eye  strain,  glaucoma,  etc.,  should 
present  little  difficulty. 

Treatment. — The  treatment  of  the  migraine  attack  is,  for  the  most 
part,  fairly  satisfactory.  There  are  few  patients  for  whom  some 
relief  cannot  be  obtained,  both  with  reference  to  the  diminution 
in  the  number  of  attacks,  and  to  the  mitigation  of  the  severity  of  the 
attacks  themselves.  The  migraine  habit,  constitution,  or  liability — 
call  it  what  one  will — exists  in  very  varying  degrees;  in  some  a  very 
slight  disturbance  is  sufficient  to  set  free  those  forces  which  culminate 
in  an  attack;  for  others  it  requires  a  very  much  greater  maladjustment. 
If  the  general  reflex  vascular  hypothesis  be  taken  as  a  tentative  explan- 
ation it  is  very  readily  understood  why  the  taking  away  of  various 
forms  of  peripheral  irritation  may  result  in  eliminating  one  or  more, 
and  in  certain  -instances  all,  of  the  causes  which  set  the  migraine 
reaction  in  operation. 


118  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

It  is  folly  to  shut  one's  eyes  to  the  very  evident  clinical  fact  that  a 
few  migraines  are  relieved,  if  not  entirely  wiped  away,  by  the  correction 
of  some  peripheral  disorder,  sometimes  more  than  one,  which  has  had 
definite  effect  on  the  nervous  system.  Just  what  the  interrelation 
may  be  between  the  severity  of  the  irritant  and  the  mildness  of  an 
attack  it  is  impossible  to  judge,  but  certainly  the  relief  from  eye 
strain,  from  diseased  turbinates,  from  adenoids,  from  constipation, 
from  dysmenorrhea,  from  a  number  of  minor  yet  definite  peripheral 
irritations,  will  relieve  a  certain  number  of  patients.  Perhaps  they 
are  the  very  slight  migraines,  perhaps  not;  one  is  not  j'^et  in  a  position 
to  say.  One  should,  therefore,  eliminate  at  the  onset  such  of  these 
structural  defects  as  are  shown  to  have  some  influence  on  the  nervous 
system.  In  denying  any  possibility  to  these  influences  in  the  causation 
of  a  migraine  attack,  one  errs  as  badly  as  when  maintaining  some  one 
of  them  to  be  the  only  and  ijivariable  element  in  the  case,  as  faddists 
are  doing  and  always  have  done. 

Gastro-intestinal  factors  are  closely  analogous  to  those  just  men- 
tioned. In  the  minds  of  most  clinicians,  and  certainly  as  generalized 
in  the  feelings  of  those  most  affected,  it  is  in  the  stomach,  liver,  or 
intestines  that  the  main  seat  of  the  trouble  is  to  be  sought.  The 
gastro-intestinal  factor  is  undoubted  in  many  cases;  it  may  be  exclu- 
sively gastric  or  colonic;  perverted  chemism,  perverted  bacterial  action 
(primary  or  secondary  factors,  no  one  can  yet  say).  As  to  the  sig- 
nificance of  chemical  features,  resulting  from  altered  gastric  secretions 
or  from  toxic  bacterial  products,  we  are  entirely  in  the  dark.  It  is 
certain  that  none  of  the  products  which  have  been  held  responsible 
as  auto-intoxicants  are  universal  causes.  At  any  rate,  the  general 
features  of  gastro-intestinal  hygiene  should  be  carried  out.  Constipa- 
tion is  to  be  avoided,  and  such  diet  taken  as  experience  has  shown 
is  individually  applicable.  Excesses  in  certain  articles  of  diet  are  held 
by  many  as  exciting  causes;  such  empirical  feelings  should  be  re- 
spected; the  patient  often  knows  himself  better  than  does  the 
physician. 

In  some,  excessive  carbohydrate  intake  acts  disastrously;  in  others 
wine,  whisky,  or  gin.  The  history  of  inability  to  eat  fatty  food,  par- 
ticularly sausages,  is  not  infrequent. 

In  rarer  instances,  one  notes  that  certain  auditory  stimuli  may 
bring  on  a  migraine.  To  attend  certain  fatiguing  and  thrilling  operas 
is  followed  in  some  by  migraine  attacks.  Here  psychical  mechanisms 
are  at  work. 

If  the  varying  elements  mentioned  have  any  real  relation,  it  is 
evident  why  such  a  variety  of  measures  will  be  of  help  to  a  few,  and 
why  so  many  more  will  be  worthless  for  many  but  useful  for  some. 
Medication  between  attacks  is  largely  useless,  save  naturally  in  the 
symptomatic  migraines.  General  medication,  for  no  definite  purpose 
but  just  in  the  hope  that  it  may  do  good,  as  iodides,  bromides, 
strychnine,  etc.,  is  senseless.     If  definite  factors  are  found  that  need 


OPHTHALMIC  MIGRAINE  119 

correction,  and  can  be  so  modified  by  drugs  in  the  desired  direction, 
then  they  will  prove  useful.  Thus  iodides  will  undoubtedly  help 
many  presenile  arteriosclerotic  migraines;  bromides  are  useful  for 
sleepless  and  irritable  conditions  which  provide  a  good  foundation  for 
the  nervous  instability  that  permits  an  attack;  laxatives  are  called  for 
if  persistent  constipation  bears  any  causal  relationship. 

Complicated  systems  of  diet  have  been  devised.  Usually  such  are 
more  prolific  in  engendering  semi-invalidism  than  useful  for  migraine. 
Here  and  there  a  patient  derives  benefit  from  a  strict  dietary  regime, 
but  unless  there  are  real  reasons  why  a  patient  should  not  eat  red 
meat,  or  tomatoes,  or  sundry  other  articles,  as  determined  by  actual 
experience  and  under  repeated  experimental  trials,  in  order  to  eliminate 
faddist's  errors,  the  patient  is  better  off  without  a  diet  card.  The 
reasons  sought  for  are  not  those  contained  in  many  treatises  on 
dietetics,  in  which  primitive  notions  concerning  difi^erences  in  red  meat 
and  white  meat,  vegetables  growing  under  the  ground  and  those  above 
the  ground,  are  foolishly  perpetuated.  The  only  satisfactory  manner 
to  attack  the  metabolic  problem  is  to  carry  out  a  complete  metabolism 
analysis.  Haphazard  attacks  here  and  there  lead  only  to  premature 
and  insecure  judgments. 

Complete  formulas  for  attacking  excessive  bacterial  putrefaction 
are  applicable  only  when  it  is  proved  that  such  excessive  bacterial 
action  exists  and  has  a  relation  to  the  migraine.  The  hypothesis 
cannot  be  excluded  ex  cathedra,  but  it  remains  unproved  for  most 
cases,  and  of  doubtful  applicability  in  a  few.  The  belief  that  the 
presence  of  indicanuria  is  an  infallible  index  of  harmful  putrefactive 
products  is  not  well  founded. 

The  avoidance  of  alcohol  and  tobacco,  while  advisable,  is  so  only 
relatively.  The  individual's  reaction  to  all  influences  should  be 
rigidly  estimated  before  those  usually  self-evident  restrictions  are 
imposed  in  the  name  of  health. 

In  certain  individuals  a  change  of  occupation  may  be  absolutely 
necessary,  but  here  again  one  must  be  wisely  conservative,  and  not 
consign  all  migraine  patients  to  an  outdoor  life,  especially  when  outdoor 
workers  are  by  no  means  exempt  and  ought  to  be  clerks.  The  character 
of  the  work  is  to  be  borne  in  mind.  The  elements  of  haste,  of  pressure, 
and  of  lack  of  leisure  are  to  be  thought  of  in  this  connection. 

Psychoanalysis  should  be  advised  for  severe  recurrent  migraines  in 
neurotic  individuals.  Chronic  headaches  are  usually  psychogenic  in 
origin  and  need  psychoanalysis. 

For  the  treatment  of  the  attack  itself,  one  finds  that  a  like  fitting 
of  remedies  to  the  individual  is  called  for.  In  the  initial  phase  of 
vasoconstriction  a  number  of  vasodilators  are  of  service,  although 
their  action  is  extremely  unequal.  The  nitrites  and  nitrates  have  been 
employed  for  years,  and  usually  with  a  fair  degree  of  success  if  the 
dosage  and  individual  member  of  the  group  be  correctly  chosen  with 
reference  to  the  severity  of  the  attack.     A  mixture  is  of  greatest  value; 


120  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

nitroglycerin  and  erythrol  tetranitrate  give  the  best  combination,  for 
following  the  very  evanescent  and  powerful  action  of  the  former,  the 
more  prolonged  and  steady  action  of  the  latter  maintains  the  effect. 
The  slowly  acting  nitrites  are  practically  useless.  Nature's  readjust- 
ment, vasodilatation  by  vomiting,  etc.,  has  already  reduced  the  cerebral 
pressure,  and  the  stage  has  passed  when  the  dilating  remedies  might 
be  useful.  It  is  practically  only  in  the  vasoconstriction  stage  that  the 
nitrites  are  worth  much;  and  in  many  they  are  inefficient,  the  reasons 
for  this  being  as  yet  unappreciated.  Given  too  late,  they  overdo 
the  dilatation  and  increase  the  difficulty. 

The  analgesic  vasodilators  have  come  to  occupy  the  front  rank. 
The  precise  significance  of  each  must  be  appreciated  in  order  to  obtain 
the  best  results.  Solubility,  time  of  absorption,  slight  differences  in 
the  chemical  formula  and  in  action,  continuance  of  effect  with  minimum 
by-effects,  are  all  to  be  studied.  The  list  is  a  long  one  and  is  constantly 
on  the  increase.  Antipyrin,  acetanilid,  phenacetin,  and  the  related 
salicylic  acid  (aspirin,  etc.)  compounds  are  the  chief  members.  It  is 
to  be  remembered  that  while  their  general  action  is  closely  related, 
there  are  specific  differences  in  the  working  of  each,  and  the  measure 
of  success  that  one  has  in  mastering  the  majority  of  migraines  depends 
upon  a  knowledge  of  these  factors.  Antipyrin,  by  reason  of  its  rapid 
solubility  and  quick  action,  occupies  an  important  place,  but  is  not 
always  applicable.  Acetanilid,  alone  or  in  combination  with  other 
analgesics  of  related  type  (salicylic  acid  derivatives),  bromides,  and 
caffeine,  are  also  valuable.  The  dosage  should  be  graded  according 
to  the  usual  severity  of  the  attacks.  Tolerance  is  established  in  the 
quickly  recurring  attacks,  and  changes  must  be  made.  It  is  not  yet 
certain  what  part  is  played  by  the  respective  analgesic  and  vasodila- 
tation actions  of  this  group.  They  have  robbed  migraine  of  most  of 
its  terrors,  and  tended  to  diminish  the  use  of  morphine  and  its  deriva- 
tives very  markedly. 

Caffeine  is  a  much  overrated  drug.  In  the  abortive  attacks  and  in 
the  morning  remains  of  a  migraine  it  is  useful;  but  for  a  full-fledged 
attack  it  is  not  efficient.  Similarly,  bromides  alone,  chloral,  and  other 
widely  used  drugs  are  valuable  only  in  mild  attacks.  They  should  be 
used  in  preference  to  other  more  potent  remedies,  which  should  be 
reserved  for  the  severer  attacks,  in  order  that  one's  therapeutic  meas- 
ures may  more  correctly  approximate  the  needs  of  each  individual 
occasion. 

The  use  of  aconite  and  Cannabis  indica  is  more  restricted  now  that 
really  efficient  analgesics  are  known.  Aconite  is  rarely  called  for, 
while  Cannabis  indica  or  Cannabis  americana  has  a  limited,  though 
no  less  definite,  place.  In  attacks  associated  with  much  mental 
depression  the  addition  of  cannabis  is  often  useful.  The  often  experi- 
enced inefficacy  of  this  latter  remedy  is  largely  due  to  its  extreme 
variability^     Great  care  is  therefore  to  be  exercised  in  the  selection  of  a 


PERIODIC  PALSIES  121 

proper  preparation.  Tablet  preparations  are  usually  worthless.  This 
is  equally  true  of  the  volatile  nitrite  preparations.  Opium,  or  its 
main  derivative  morphine,  should  be  used  only  as  a  last  resort.  It  is 
rarely  really  needed. 

Lying  down  in  a  quiet,  darkened  room — a  brisk  saline  laxative  taken 
as  early  as  possible,  the  patient  being  undressed  and  well  covered — 
these  are  essential  in  the  severe  exhausting  attacks.  A  very  hot  bath 
often  aids  very  materially  in  restoring  the  patient  to  comparative 
freshness.     Cold  is  to  be  avoided. 

The  greatest  folly  of  all  is  to  treat  all  patients  and  every  attack 
alike. 

Periodic  Palsies :  Ophthalmoplegic  Migraine. — It  is  known  that  in 
the  ordinary  attack  of  ophthalmic  migraine  there  may  occur  various 
sensory  or  motor  phenomena,  among  which  anesthesias  or  paralyses 
are  the  most  marked.  These  sensory  and  motor  changes  are  ex- 
tremely diverse  when  the  entire  range  of  the  migraine  symptom- 
atology is  brought  into  review,  but  there  is  one  symptom  grouping 
which,  by  reason  of  its  comparative  frequency  and  close  similarity, 
was  set  apart  from  others  occurring  in  this  affection  and  named 
by  Charcot  ophthalmoplegic  migraine,  in  order  to  distinguish  it 
from  its  more  classical  relation.  It  consists  in  a  paresis  or  a  par- 
alysis of  one  or  more  muscles  of  the  eye,  innervated  chiefly  b}'^  the 
oculomotorius,  which  comes  on  either  following  or  during  a  migraine 
attack. 

Inasmuch  as  oculomotor  pareses  or  paralyses  may  occur  from  a 
great  diversity  of  causes,  apart  from  a  migraine,  and  may  appear 
periodically,  it  has  been  held  by  many  that  the  term  ophthalmoplegic 
migraine  has  no  particular  right  to  exist,  but  the  evidence  is  too  great 
to  eliminate  migraine  as  a  competent  producing  cause  for  these  periodic 
oculomotor  paralyses. 

It  is  purposed  also  to  enlarge  the  group  and  include  the  so-called 
periodic  palsies,  familial  as  well  as  those  in  which  the  hereditary  factors 
are  not  known  to  be  present. 

Etiology. — Whether  heredity  plays  any  greater  part  here  than  in 
migraine  in  general  is  difficult  to  decide.  Certain  periodic  palsies 
show  marked  heredity. 

There  has  been  much  speculation  concerning  the  central  or  peripheral 
nature  of  this  third  nerve  palsy.  The  present  view  taken  for  migraine 
in  general,  that  it  is  due  to  a  disturbance  in  cerebral  pressure,  secondary 
to  vascular  modifications,  is  sufficient  to  account  for  the  oculomotor 
palsies  as  well,  in  view  of  the  location  of  the  peripheral  branches  of  the 
third  nerve  in  regard  to  the  cerebral  vascular  plexuses.  In  fact,  the 
occurrence  of  the  ophthalmoplegic  type  is  one  of  the  strong  arguments 
for  the  general  pressure  hypothesis,  as  Spitzer  has  well  argued.  If, 
as  has  been  shown  by  several  autopsies,  to  these  considerations  addi- 
tional local  causes  be  added,  which  increase  or  permanently  maintain 


122  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

such  pressure  effects,  the  interpretation  is  comparatively  simple. 
Thus,  exudates,  fibrous  processes,  swelling  in  the  cavernous  sinuses, 
swelling  of  the  hypophysis,  tiimor  formation,  gummata,  etc.,  have 
been  found  in  patients  suffering  from  periodic  oculomotor  paralyses 
associated  with  migraine. 

It  is  true  that  some  of  these  are  to  be  interpreted  as  symptomatic 
migraines,  in  which  the  foreign  body  acts  primarily  as  an  irritant 
to  cause  the  vascular  disturbance,  which  sets  free  the  migraine  reaction, 
and  secondarily  serves  as  an  additional  cause  of  pressure  to  bring  about 
the  palsy.  In  a  personally  observed  case,  with  basal  gummata,  the 
periodic  oculomotor  palsy  and  migraine  attacks  have  occurred  for 
a  period  extending  over  four  or  five  years,  usually  with  every  men- 
strual period.  Here  were  three  interplaying  factors,  and  the  exact 
part  played  by  each  can  only  be  inferred.  The  slight  disturbance  of 
menstruation,  usually  adjusted,  in  this  case  by  reason  of  the  exudate 
was  not.  A  migraine  was  set  up,  the  acute  pressure  of  which,  added 
to  that  of  the  exudate,  caused  the  ophthalmoplegia.  This  ophthal- 
moplegia has  become  fairly  persistent  in  the  intermigrainous  interval 
in  recent  years. 

Symptoms. — Leaving  aside  for  the  moment  the  atypical  and  symp- 
tomatic periodic  oculomotor  paralyses  due  to  other  causes  than 
migraine,  one  finds  in  these  patients,  usually  during  or  after  a  severe 
attack  of  unilateral  migraine,  with  headache,  nausea,  vomiting,  etc., 
a  ptosis  of  the  eyelid  on  the  same  side,  and  a  loss,  partial  or  complete, 
of  the  upward,  downward,  and  inward  movements  of  the  eye  of  the 
same  side.  This  eye  is  usually  directed  outward  and  downward,  and 
the  patient  sees  double.  This  may  or  may  not  be  accompanied  by 
sensory  disturbances  in  the  superior  branch  of  the  trigeminus,  just  as 
may  be  observed  in  ordinary  ophthalmic  migraine. 

After  a  variable  length  of  time,  a  few  days,  a  week  or  more,  the 
paralysis  disappears,  usually  gradually,  and  the  patient  suffers  no 
inconvenience  from  the  ocular  palsies  or  the  ptosis.  In  some  individ- 
uals such  palsies  accompanying  a  migraine  have  come  on  comparatively 
young  in  life,  almost  with  the  beginning  of  the  migraine  attacks; 
for  the  majority,  however,  they  follow  several  years  after  the  estab- 
lishment of  a  migraine,  in  some  instances  as  late  as  thirty  years.  In 
some  only  a  very  severe  attack  will  be  accompanied  by  the  oculomotor 
signs,  or  only  slight  palsies;  transitory  ptosis  may  occur  frequently. 
But  in  others  the  palsies  develop  with  each  attack  of  migraine  and 
often  in  increasing  severity.  The  effects  may  persist  longer  and  longer 
between  the  attacks,  until  in  a  few  they  become  permanent  palsies. 
This  type,  however,  often  permits  of  other  interpretations. 

A  double  lesion  can  be  understood,  although  it  rarely  occurs.  Iso- 
lated abducens  palsy  has  been  described,  also  isolated  trochlearis; 
and  complete  ophthalmoplegia  is  reported  in  a  single  case,  but  in  view 
of  the  many  possible  contributory  factors  it  perhaps  is  preferable  to 
view  such  a  case  from  another  standpoint. 


ANGIONEUROTIC  EDEMA  123 

Some  periodic  iMlsies  belong  in  this  group.  Those  palsies  occurring 
in  cranial  nerves  become  associated  intellectually  with  the  migraines 
because  of  the  accompanying  headaches,  but  the  cause  is  a  vasomotor 
disturbance.  A  similar  vasomotor  disorder  in  the  cord,  however, 
will  produce  a  periodic  paralysis  in  other  muscle  groups.  It  is  there- 
fore largely  a  matter  of  terminology  just  how  to  consider  the  periodic 
palsies.  A  more  extended  discussion  of  them  will  be  found  in  the 
vasomotor-irritability  group. 

Diagnosis. — Every  patient  should  be  regarded  as  one  suffering  from 
something  more  than  the  migraine,  until  all  accessory  causes  are 
excluded.    .  What  these  may  be  have  been  mentioned  already. 

Treatment. — Little  needs  to  be  added  to  the  therapy  outlined  under 
migraine.  Syphilis  as  a  cause  for  both  a  migraine  and  an  exudate 
should  be  treated,  and  the  Wassermann  reaction  utilized  to  clear  up 
the  diagnosis  and  therapeutic  indications. 

3.    Vasomotor  Irritability  Group. 

Angioneurotic  Edema. — This  condition  is  better  described  as  Acute 
Circumscribed  Edema  (Quincke),  since  such  a  name  does  not  commit 
one  to  its  being  a  vascular  neurosis,  although  this  is  probable. 

These  most  striking  skin  edemas  were  described  as  early  as  1778 
by  Salpertus.  Erichton  in  1801  also  observed  them,  and  Graves,  who 
gave  such  an  excellent  outline  of  exophthalmic  goitre,  in  1848,  described 
a  patient  with  localized  swelling  of  the  face,  forehead,  and  eyes,  in 
whom  the  edema  persisted  onl}^  a  few  hours.  Various  aberrant 
localizations  have  been  described  often  under  different  names.  Natur- 
ally hysteria  bulked  large  in  the  diagnosis  in  the  earlier  days.  Other 
synonyms  indicate  under  what  different  rubrics  it  was  grouped: 
urticaria,  urticaria  oedematosa,  epidermolysis  bullosa,  urticaria  tube- 
rosa,  urticaria  gangrenosa,  giant  urticaria,  all  indicate  where  one 
should  search  the  early  literature.  Further,  one  finds  rheumatic 
edema,  arthritic  edema,  repeating  rheumatic  edema,  hydrops  articu- 
lorum,  intermittent  rheumatic  edema,  neuroarthritic  edema,  in  the 
period  when  the  cases  were  grouped  among  the  "rheumatisms." 
Gastrosuccorrhea  periodica  is  a  stomach  localization.  Probably 
there  are  others  of  obscure  nature.  Acute  brain  swelling,  meningitis 
serosa,  spinal  swelling,  local  transient  edema,  intermittent  edema, 
and  periodic  paralysis  are  among  them. 

Quincke,  in  1882,  described  it  as  acute  circumscribed  skin  edema, 
while  in  a  Kiel  dissertation,  one  of  his  students,  Dinkelacker,  brought 
together  may  of  the  older  descriptions,  and  showed  the  unity  of  several 
apparently  dissimilar  processes.    He  termed  it  acute  edema. 

Occurrence. — The  disorder  is  not  frequent,  yet  it  is  not  rare.  Men 
and  women  appear  about  equally  involved.  It  may  be  present  in 
young  children — one  and  a  half  months  (Crozier,  Griffith);  three 
months  (Dinkelacker).     After  forty  it  appears  very  rarely,  as  an 


124 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


initial  development,  although  in  affected  individuals  it  may  persist 
until  late  in  life.  Cassirer  reported  cases  of  seventy-nine  and  sixty- 
nine,  in  which  the  disease  appeared  comparatively  late  in  life.  Raven 
reports  a  case  in  a  woman  of  eighty-six. 

Occupation  apparently  plays  no  role.  Heredity  on  the  other  hand 
is  conspicuous.  Many  authors  have  mentioned  this  feature.  Osier's 
family  tree  has  been  freely  cited,  and  is  here  reproduced  in  slightly 
changed  form.  Ensor  reports  a  family  of  eighty  members,  with 
thirty-three  affected  individuals  twelve  of  whom  died  of  edema  of  the 
glottis.  Similar  hereditary  features  are  reported  by  several  observers. 
The  question  of  its  transmission  has  not  been  completely  cleared  up. 
In  Apert  and  Delille's  families  only  the  males  were  affected,  but  this 
does  not  seem  to  be  the  rule. 


INHERITANCE  IN  ANGIO-NEUROTIC   (XDEMA 
"T"  FAMILY 


n        67^ 


■O 


III 


IV 


£Wi  S 


"i       ^     i 


*     46i44*4i4 


£%  6^ 


n  "ALE  I  I 

O  female)  o 


Fig.  44. — Chart  showing  heredity  in  an  angioneurotic  edema  family.      (Osier.) 


In  many  families,  similar  types  of  localized  edema  prevail  in  the 
members,  while  in  others,  apparently  more  often,  all  of  the  possible 
variants  disappear.  Other  nervous  system  involvements  appear 
associated  with  many  of  the  families;  how  much  of  this  is  largely 
coincidence,  how  much  general  neuropathic  causal  relationship  is 
difficult  to  determine  from  the  studies  at  hand. 

The  syndrome  is  associated  infrequently  with  tabes,  myasthenia 
gravis,  spinal  cord  tumor,  exophthalmic  goitre,  myxedema,  periodic 
palsies,  while  it  seems  very  frequently  associated  with  many  so-called 
functional  neuropathic  states — hysteria,  neurasthenia,  tics,  compulsion 
neuroses,  migraine,  etc. — and  in  certain  psychotic  individuals  with 
schizophrenia,  manic-depressive  psychosis,  feeble-mindedness. 

Local  traumata  play  a  role  at  times,  particularly  in  determining 
the  location  of  the  swelling.     Emotional  shock  bulks  large  as  a  direct 


ANGIONEUROTIC  EDEMA  125 

etiological  factor,  as  does  also  the  action  of  thermal  influences.  Cold 
is  very  freciuently  an  exciting  factor  in  the  reaction.  Menstrual 
factors  seem  to  enter  into  the  etiology  of  certain  cases. 

A  moment's  reflection,  therefore,  will  show  that  under  the  term 
Acute  Circumscribed  Edema,  one  is  dealing  with  phenomena  of  great 
variability  and  multiform  genetic  pathogeny.  In  discussing  the 
pathology,  a  return  will  be  made  to  this  many-sided  etiology. 

Symptoms.^ — The  original  conception  of  Quincke  has  been  much 
employed,  and  Cassirer  in  his  large  monograph  shows  the  present- 
day  trend  to  include  a  large  number  of  acute  edematous  swellings 
within  the  nosological  group.  Thus  one  distinguishes  localized  edema 
of  the  skin,  edemas  of  the  mucous  membrane,  of  the  eyelids,  mouth, 
glottis,  esophagus,  stomach,  intestines,  respiratory  tract;  edemas  of 
the  joints,  the  meninges,  the  tendinous  aponeuroses,  of  the  spinal 
cord,  of  the  brain,  of  the  kidneys,  with  polyuria,  albuminuria,  hemo- 
globinuria, diminished  secretions,  and  edema  of  other  structures. 

The  onset  is  usually  acute,  with  some  initial  prodromal  signs  of 
malaise,  fatigue,  chilliness,  anorexia,  nausea,  and  slight  rise  in  tem- 
perature. The  symptoms  that  develop  will  depend  upon  the  localiza- 
tion of  the  process. 

In  the  skin  there  are  isolated  swellings.  These  are  localized,  variable 
in  size,  at  times  small,  resembling  urticarial  blotches  (intermediary 
forms)  but  usually  as  distinct  swellings,  with  an  elastic  feel,  and  due 
to  local  accumulations  of  clear  serum  within  the  skin.  The  color 
of  the  swelling  is  usually  that  of  the  skin,  or  paler,  rarely  red  or 
reddish.  The  swelling  comes  on  with  great  rapidity,  a  few  moments 
only,  and  remains  a  few  hours,  mostly  a  few  days,  and  then  disappears 
without  leaving  any  trace.  They  are  as  a  rule  non-irritating,  painless, 
and  only  cause  discomfort  as  a  result  of  the  tension.  Certain  cases 
show  burning,  itching,  and  intense  pain. 

The  size  of  the  edematous  patches  varies  greatly.  At  times  very 
small — one-half  inch — they  are  more  apt  to  be  three  to  four  inches  in 
diameter,  or  at  times  involve  the  larger  part  of  a  limb.  The  scrotum 
may  at  times  swell  up  to  the  size  of  a  foot-ball.  The  penis,  in  cases 
reported  by  Borner,  has  swollen  to  double  its  diameter.  The  entire 
body  was  swollen  also  in  a  remarkable  case  reported  by  Diethelm. 
At  times  the  swellings  are  numerous,  polymorphous,  semi-confluent. 
They  rarely  rise  more  than  one-quarter  to  one-half  centimeter,  but 
swellings  two  to  four  inches  above  the  skin  occur.  The  margins 
of  the  swellings  are  usually  sharply  circumscribed,  but  at  times  may 
shade  off  imperceptibly  into  normal  areas.  The  usual  descriptions 
of  the  swellings  are  circular  or  sausage-shaped.  The  swellings  invade 
almost  any  layer  in  the  skin,  the  musculature,  or  they  may  even 
invade  the  periosteum.     Some  have  been  termed  pseudolipomata. 

The  consistency  is  semi-solid,  non-pitting,  or  slightly  so.  The  color 
as  stated  is  usually  that  of  the  normal  skin,  or  it  may  be  paler,  or  have  a 
cadaveric  hue.     Again  it  is  pinkish  to  red,  or  even  deep  red.     Often 


126  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

the  color  disappears  on  pressure.  The  color  may  change  during  the 
rise  of  the  swelling. 

Local  temperature  varies.  At  times  the  skin  is  colder,  again  it  is 
warmer  than  that  of  the  non-affected  parts.  Exact  studies  are  want- 
ing. It  seems  not  unlikely  that  there  is  an  initial  increase  in  the 
local  temperature. 

Sensory  changes  are  not  present  as  a  rule.  Certain  cases  have 
shown  preliminary  neuralgic  twinges,  no  definite,  sensory  defect 
has  been  noted,  but  refined  methods  of  examination,  such  as  those 
demanded  by  Head,  have  not  yet  been  made.  There  is  frequently 
the  subjective  sense  of  great  discomfort,  especially  in  marked  swellings 
about  the  face. 

There  are  rarely  any  residuals,  although  occasionally  scaling  or 
peeling  has  been  observed,  probably  for  the  more  superficially  lying 
edemas. 

Secretory  symptoms  have  not  been  carefully  recorded.  Local 
hyperidrosis,  dermatographia,  increased  tear  secretion  have  been 
noted. 

The  location  of  the  swelling  may  be  almost  anywhere,  it  cannot 
be  said  that  one  place  more  than  another  is  a  favorite  site  (statistically). 
Exposed  portions  of  the  body  seem  to  be  more  often  involved,  but 
when  on  the  hand  or  hands,  the  distribution  is  not  of  the  glove  type, 
nor  are  the  swellings  apt  to  be  symmetrical,  nor  do  they  seem  to  follow 
radicular  or  peripheral  distributions.  There  is  a  distinct  tendency 
for  a  recurring  edema  to  occupy  the  position  involved  during  a 
former    attack. 

Periarticular  swelling  constitutes  a  peculiar  type,  so  do  also  parotid 
and  salivary  gland  edemas. 

■Mucous  Membranes. — These  are  frequently  involved.  The  lips, 
mouth,  soft  palate,  tongue,  pharyngeal  pillars,  nasal  membrane, 
larynx  are  all  sites  of  election.  The  last  is  particularly  frequent 
and  is  dangerous  to  life.  In  these  cases,  other  structures  than  the 
larynx  are  implicated,  especially  the  epiglottis  and  closely  associated 
structures.  In  the  larynx  the  mucous  membrane  is  swollen  and 
tense;  the  edema  infiltrates  throughout. 

When  the  larynx  is  involved,  the  symptoms  are  apt  to  be  very 
marked.  There  is  beginning  tickling,  and  rapidly  oncoming  difficulty 
in  breathing,  until  marked  dyspnea  may  supervene,  with  death, 
unless  intubation  or  tracheotomy  is  performed.  Some  of  these  pa- 
tients die  within  a  few  hours.  Many  cases,  on  the  other  hand,  clear 
up  in  an  hour,  after  severe  dyspneic  symptoms.  Acute  conjunctival 
edema  is  not  infrequent. 

Edemas  within  the  bronchi  occur  in  perhaps  twenty  per  cent,  of 
the  cases.  They  make  up  a  certain  percentage  of  the  cases  of  asthma. 
Certain  hay  fevers  possibly  belong  in  this  group.  Lung  edemas  have 
been  described. 

In  edemas  of  the  stomach  (gastrosuccorrhea  periodica)  external  signs 


ANGIONEUROTIC  EDEMA  127 

are  also  usually  present.  There  may  be  intermittent  vomiting',  or 
sudden  acute  pains  and  anorexia.  The  attack  may  last  a  few  hours 
with  severe  pain,  and  finally  more  or  less  continuous  vomiting  of  clear  or 
bile-colored  watery  masses,  marked  thirst,  and  gradual  disappearance 
of  all  of  the  symptoms.  Bits  of  gastric  mucosa  have  been  accidentally 
dislodged  which  showed  marked  edematous  swelling. 

In  intestinal  localizations  profuse  diarrheas  are  present,  with  colicky 
pains,  meteorism,  tenderness  of  the  abdomen,  diminished  urination, 
great  thirst,  and  collapse.  The  diarrheas  are  purely  nervous  diarrheas, 
so-called,  and  occur  in  association  with  other  signs  of  a  circumscribed 
edema. 

Rarer  localizations  present  in  the  tendons  have  been  described, 
particularly  by  Schlesinger.  Muscle  edemas  are  also  rarely  described, 
although  it  is  probable  that  they  are  of  frequent  occurrence.  Articular 
edemas  have  been  mentiond.  They  are  frequently  of  psychical  ori- 
gin.    Compare  gout  and  anger. 

Optic-nerve  edema  is  one  of  the  rarer  localizations,  as  is  also  an 
edema  in  the  labyrinth  leading  to  a  Meniere  syndrome. 

The  bladder,  kidney,  and  heart  structures  are  among  the  rarest 
localizations.  Meningitis  serosa,  aphasia,  are  among  some  of  the 
more  problematical  occurrences  reported  and  periodic  paralysis  and 
myasthenia  gravis  are  included  here  as  well  as  in  the  previous  group 
allied  to  the  migraines. 

Prognosis. — In  general  this  is  not  good.  The  tendency  to  laryngeal 
localization  must  always  be  viewed  with  gravitv.  A  great  many 
individuals  have  died  from  edema  of  the  glottis.  Remissions  are  to 
be  expected.  Some  patients  suffer  many  years,  others,  but  the  min- 
ority it  would  appear,  have  but  few  attacks.  There  is  some  general 
tendency  for  the  disorder  to  become  milder  as  the  affected  individual 
grows  older. 

Transition  forms  are  common,  especially  urticaria-like  eruptions. 
Acroparesthesias,  Raynaud-like  attacks,  local  asphyxias  of  the  extrem- 
ities, paroxysmal  hemoglobinuria,  acroasphyxia  chronica,  erythro- 
melalgia,  periodic  paralysis,  epidermolysis  bullosa  hereditaria,  synovial 
serositis,  fibrous  serositis,  herpes  zoster  are  all  affections  with  which 
attacks  have  been  combined,  singly  or  in  groups  of  two  or  three.  Occa- 
sionally edema,  acroparesthesia,  and  erythromelalgia  may  alternate 
in  one  and  the  same  patient. 

Pathogenesis. — Our  conceptions  concerning  edema  are  undergoing 
such  vital  modifications  that  it  is  practically  impossible  to  interpret 
the  findings  here  outlined  along  those  present-day  lines  that  regard 
all  edemas  as  cell  phenomena  and  independent  of  the  mechanical 
conceptions  of  stasis,  pressure,  osmotic  tension  of  the  vascular  and 
lymph  vessels,  etc.  The  studies  of  edema  made  by  Fischer  and  others 
emphasize  the  purely  physico-chemical  side  of  the  problem.  They 
neglect  the  role  of  the  vegetative  system  in  regulating  tissue  tension 
and  cellular  chemism.     The  simple  statement  that  the  disorder  is 


128  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

an  angioneurosis  by  no  means  clears  the  situation,  although  it  is 
certain  that  the  sympathetics  are  media  from  cause  to  effect.  The 
study  of  anaphylactic  phenomena,  especially  as  seen  in  the  so-called 
anaphylactic  serum  reactions,  or  serum  diseases,  has  offered  suggestive 
glimpses  indicating  certain  analogies  with  the  series  of  changes  here 
outlined.  Wherein  are  the  proteids  supposed  to  cause  these  related 
to  the  endocrinous  hormones?  It  can  only  be  stated  that  precisely 
similar  processes  and  appearances  are  found  in  the  serum  reactions, 
and  that  it  is  not  without  profit  to  enquire  more  into  the  mechanism 
of  their  production  in  an  attempt  to  understand  acute  circumscribed 
edema.  Unfortunately  the  mechanisms  of  the  changes  in  the  ana- 
phylactic reactions  are  still  much  in  the  dark.  There  is  a  distinct 
tendency  to  include  the  anaphylactic  reactions  under  the  phenomena 
regulated  by  the  vegetative  nervous  system.^ 

Acute  circumscribed  edema,  has  also  been  interpreted  as  a  modified 
colloid  absorption  reaction,  due  to  toxic  influences  brought  to  the  cells 
of  the  deeper  layers  of  skin,  muscle  or  mucous  membrane.  The  view 
here  tentatively  adopted  is  that  it  is  a  neural  reaction  brought  about 
through  the  vegetative  nervous  system,  which  controls  reciprocal 
tension  relations,  or  cellular  chemical  composition  relations. 

It  is  not  improbable  that  there  are  a  series  of  reactions  represented 
in  the  acute  circumscribed  edemas.  It  is  no  unicum,  and  analysis 
will  show  that  a  number  of  different  pathological  processes  may  underly 
precisely  similar  phenomena,  be  they  in  any  vascular  area  of  the 
body. 

Cassirer  adopts  this  viewpoint,  but  consents  to  make  only  two  groups 
of  cases — (a)  a  toxic,  autotoxic  group,  in  which  the  poison  works  in 
some  mysterious  way,  which  a  wealth  of  language  can  conceal,  better 
than  it  can  reveal,  and  (6)  a  heredofamilial  or  constitutional  neuro- 
pathic group,  which  he  regards  as  intimately  associated  with  instability 
in  certain  parts  of  the  vegetative  nervous  system.  This  may  be,  he 
says,  associated  in  some  manner  with  modifications  in  the  internal 
gland  secretions.  Here  we  enter  another  dark  portal.  At  all  events, 
Cassirer  is  loath  to  permit  so-called  angioneurotic  edema  to  wander 
from  the  neurological  fold,  and  concludes  that  the  disease  is  con- 
ditioned— at  least  his  group  (b) — by  the  lability  of  the  vegetative 
nervous  system. 

Treatment. — This  is  purely  empirical.  It  consists  first  in  avoiding 
all  those  things  which  experience  has  shown  to  be  liable  to  bring  on  an 
attack. 

If  one  has  one  of  the  more  pronounced  toxic-anaphylaxis-like  react- 
ing types,  careful  study  must  be  made  of  all  of  the  patient's  protein 
reactions,  and  attempts  made  calculated  to  regulate  the  diet  accord- 
ingly. It  seems  plausible  that  it  is  through  the  gastro-intestinal  canal 
that  such  products  gain  entry,  particularly  in  food,  yet  some  may 

1  Roncoroni,  Ergebnisse  d.  Neurologic  u.  Psychiatrie,  vol.  ii,  No.  1. 


ANGIONEUROTIC  EDEMA  129 

enter  the  respiratory  tract,  as  seems  to  be  the  case  in  the  related  hay- 
fever  reactions  which  are  known  to  follow  certain  contacts,  variously 
ascribed  to  ragweed,  rose,  hay,  and  other  pollens,  or  even  the  emana- 
tions from  cattle. 

From  specific  exclusion  of  certain  proteins  one  passes  to  the  general 
hygiene  of  the  intestine.  This  means  a  sort  of  search  in  the  dark  for 
effective  agencies  by  chemical  means.  One  is  justified  notwith- 
standing in  trying  to  bring  about  altered  bowel  conditions,  which 
empirically  may  do  some  good,  when  a  laissez-faire  attitude  seems  to 
perpetuate  the  disturbance.  Naturally  one  should  avoid  intestinal 
therapy,  should  the  patient  be  of  an  entirely  different  type,  say,  the 
intensely  neurotic  forms  with  familial  hereditary  burdens,  and  emotional 
shock  reactions.    These  patients  need  a  psychoanalysis. 

Of  the  gastro-intestinal  antiseptics  so-called,  few  are  such.  Menthol, 
saline  laxatives,  carbonated  waters,  careful  dieting  (?)  may  be  found 
among  the  conventional  remedies  in  the  books.  The  taking  of  a 
milk-vegetable  diet  has  been  coincident  with  betterment  in  some 
individuals  and  coincident  with  getting  worse  in  others. 

In  certain  cases  with  associated  toxemias,  such  as  malaria,  etc.,  a 
specific  therapy  is  indicated. 

On  the  supposition  that  the  bloodvessels  needed  bracing  up  to 
prevent  transudation  through  their  walls,  also  a  hypothetical  postulate, 
apparently  inadequate,  such  drugs  as  strychnine,  ergot,  arsenic, 
atropine,  morphine  have  been  recommended.  While  all  of  these  will 
bring  about  vasoconstriction  it  is  not  apparent  whether  they  can  alter 
a  hypothetical  transudability  or  not.  Calcium  lactate  is  the  modern 
weapon  for  this  latter.  The  authors  have  not  seen  it  recommended, 
but  it  may  be  of  service  in  preventing  transudates,  as  such  are  thought 
to  be  conditioned  by  a  diminution  in  the  calcium  content  of  the  body 
plasma.  Cassirer  mentions  calcium  chlorate.  At  all  events  the  vaso- 
constricting  drugs  have  not  been  of  any  particular  service  clinically. 
Now  and  then  they  seem  of  service;  none  have  been  proven  of  pro- 
phylactic value  which  is  a  stricter  test  of  their  usefulness,  since  the 
disease  is  so  self-limited.     Atropin  is  of  great  value  at  times. 

In  many  cases  associated  with  laryngeal  symptoms,  intubation 
is  often  necessary — even  tracheotomy.  There  are  records  of  certain 
patients  condemned  to  the  persistent  use  of  the  tracheotomy  tube. 

In  the  more  strictly  neurotic  type — Cassirer's  group  (6) — it  is  impor- 
tant that  the  patient  be  taught  a  healthy  morale.  The  substitution 
of  reasonable  and  intelligent  actions  for  purely  instinctive  and  emo- 
tional reactions  must  be  acquired  by  them,  if  they  can  hope  to  in  any 
way  control  their  hair-trigger  vegetative  nervous  system.  Perhaps 
it  was  so  given  to  them,  defective  and  badly  coordinated;  even  then  a 
rational  pedagogy  will  prove  of  service.  Many  will  be  helped  by  the 
methods  outlined  by  Dubois  or  Dejerine;^  others  will  need  a  psycho- 

1  Dubois,  Psychic  Treatment  of  Nervous  Disorders;  Dejerine,  Psychoneuroses  and 
Psychotherapy,   Philadelphia,    1915. 
9 


130 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


pd' 


-ch 


analysis.  Steckel  has  reported  some  extremely  interesting  and  severe 
asthmatic  cases,  with  pronounced  symptoms  of  circumscribed  edemas 
with  psychoneurotic  combinations  or  complications.     Just  how  the 

psychical  pathways  become  involved  in  their 
complex  neurobiochemical  relations  has  been 
shown  in  the  pages  preceding  also  in  the 
chapters  on  the  neuroses  and  psychoneu- 
roses,  under  which  groups  also  many  of  these 
patients  are  classified. 

Multiple  Neurotic  Gangrene  of  the  Skin. 
- — ^The  condition  described  by  this  title  may 
f-'^^:^^^^^^^  be  mentioned.     It  is  an  analogous  process 

in  which  the  vasomotor  constriction  is  not 
limited  solely  to  the  extremities,  but  to 
br-'^^'^^Sll^d  "^  apparently  unrelated,  isolated  patches  of 
the  skin. 

While  exaggerations  in  neuropaths  (hys- 
teria), or  in  psychoses  (schizophrenia)  may 
bring  these  changes  about  by  artefact, 
malingering  explains  only  a  small  number 
of  them,  granting  that  the  term  is  any 
explanation  at  all. 

The  disorder  sets  in  with  a  burning  and 
""JO  prickling  of  the  skin.     Then  after  a  variable 

length  of  time  (a  few  minutes  to  forty-eight 
hours)  swellings  occur,  blisters  form,  with 
■'■^  later   necrosis.      At  times  only  a  circum- 

scribed edematous  bleb  forms. 

Sweat  Secretory  Mechanisms.  —  These 
are  closely  related  to,  yet  independent  of, 
the  vasomotor  mechanisms.  They  probably 
have  independent  ganglion  cell  representa- 
tion in  the  lateral  horns.  In  general  those 
pharmacological  agents  which  increase  vaso- 
dilation (autonomic)  increase  sweat  secre- 
tions, yet  vasodilation  may  occur  without 
sweating,  and  the  latter  may  occur  with 
anemia  and  vasoconstriction  (cold  sweat  of 
fear,  sweating  of  face  in  migraine,  epileptic 
aura). 

Cervical  sympathetic  and  bulbar-auto- 
nomic innervation  is  probable,  and  cortical 
connections,  though  still  topographically 
unanalyzed  are  certain  (unilateral  sweating 
in  thalamic  lesions,  hemiplegia,  hysteria, 
compulsion  neuroses,  schizophrenia).  Cer- 
tain anatomists  place  the  cortical  pathways 


Li 


Fig.  45. — Scheme  of  spinal 
centers  of  the  sweat  secre- 
tions: /,  center  for  the  face 
and  neck;  hr,  center  of  the 
upper  extremities;  pd,  center 
for  lower  extremities;  a, 
superior  cervical  ganglion;  h, 
middle  cervical  ganglion;  c, 
inferior  cervical  ganglion;  m, 
medulla.     (Bechterew.) 


PILOMOTOR  SYSTEM  131 

among  the  motor  tracts  in  the  internal  capsule.  The  hypothalamus 
is  made  a  midbrain  center  by  some.^ 

Clinically,  sweating  is  increased  in  certain  hemiplegias,  and  in  zoster. 
Diminution  of  the  sweat  is  seen  in  certain  cases  of  poliomyelitis, 
multiple  sclerosis,  syringomyelia,  myelitis  and  tumor  of  the  spinal 
cord.  Great  variability  in  the  sweat  activity  is  seen  in  many  psycho- 
pathic individuals,  in  vagotonic  types  and  in  the  psychoneuroses, 
hysteria,  anxiety  states,  compulsive  states. 

The  reactions  of  the  sweat  secretion  mechanisms  are  exquisitely 
sensitive,  as  is  seen  by  the  response  to  pain,  gastro-intestinal  cramp 
states,  action  of  carminatives,  to  nicotine,  to  anxiety  and  to  joy. 
Veraguth's  psychogalvanic  reflex  experiments  show  that  a  close 
relationship  exists  between  the  skin  secretions  and  psychical  processes 
(Jung).  The  fineness  of  registration  and  the  extreme  complexity 
of  the  phenomena,  however,  militate  against  the  practical  utility  of 
the  galvanometer  tests. 

Pilomotor  System. — The  smooth  muscle  fibers  of  the  skin  are  under 
sympathetic  innervation.  The  pilomotor  fibers  run  with  the  sensory 
fibers  (Higier),  each  sensory  nerve  carrying  fibers  from  about  five 
sympathetic  ganglia  (Higier),  and  have  similar  topographical  (seg- 
mental) distributions.  Mechanical,  thermal,  and  electrical  stimuli 
cause  contractions.  The  erector  pilee  reflexes  (best  observed  by  side 
light)  are  particularly  responsive  to  cold.  The  frequently  felt  pares- 
thesise,  acroparesthesise,  etc.,  of  psychoneurotics  depends  upon  these 
sympathetic  reactions,  and  their  exact  observation  is  of  much  diagnostic 
importance.  Mackenzie's  observations  should  be  consulted  by  the 
interested  student.^  The  feeling  of  cold  over  the  abdomen  after 
taking  cold  water  in  the  stomach  is  an  example  of  the  relationship 
of  the  sympathetic  innervation  of  an  internal  organ  and  a  skin  area.-'' 
A  large  number  of  analogous  phenomena  are  known.  Thus  among 
them,  mechanical  stimuli  of  the  plexus  pudendi,  as  in  cystitis,  rectal 
exploration,  prostatic  massage,  prostatitis,  causes  distinct  gooseflesh 
or  paresthesia  in  the  region  of  the  motor  lumbar  plexus.  Erector 
pilse  crises  are  known  to  occur  in  tabes;  they  are  at  times  migraine 
equivalents.  Psychical  stimuli  may  lead  to  localized,  or  more  often 
to  generalized  reactions.  A  great  variety  of  extremely  important 
skin  hallucinatory  experiences  are  known  to  occur  in  dementia  precox, 
and  in  the  psychoneuroses,  hysteria,  anxiety  states,  compulsive  states, 
(pathological  blushing,  etc.).  Their  correlation  is  only  just  being 
understood  through  the  results  of  psychoanalytic  research. 

1  See  Miiller  and  Gliiser,  Deut.  Zeit.  f.  Nerven.,  vol.  47-48,  p.  365  for  literature. 

2  Mackenzie,  The  Signs  of  Disease.  '  Head's  Zones,  Mackenzie. 


CHAPTER   IV. 
THE  ENDOCRINOPATHIES. 

INTERNAL  SECRETIONS. 

Medical  biology  has  not  yet  arrived  at  an  hypothesis  sufficiently 
comprehensive  to  permit  a  unitary  scheme  which  can  explain  the 
relations  of  the  vegetative  nervous  system  to  the  control  of  the  viscera. 
Some  suggestions  have  been  offered  concerning  the  action  of  the 
.vegetative  nervous  system  upon  the  gastro-intestinal  tract,  and  also 
some  ideas  relative  to  the  intricate  adjustments  of  internal  and  external 
organs  brought  about  through  the  bloodvessels  have  been  reviewed. 
The  problems  of  cellular  adjustment  now  demand  attention.  These 
concern  the  vital  phenomena  of  anabolism  and  katabolism,  and  the 
adjustment  of  all  of  the  organs  of  the  body  involved  in  the  elaboration 
of  special  substances  which  are  of  importance  to  the  metabolism  of 
the  rest  of  the  organism. 

Thus,  what  part  is  played  by  the  nervous  system  in  the  carbohydrate 
oxidations  of  the  body,  acting  chiefly  through  the  lungs,  the  liver, 
the  pancreas,  and  suprarenals?  The  substance  of  the  hypophysis, 
its  hormones  or  active  substances,  what  are  they;  and  what  is  the 
interrelationship  between  them  and  growth  as  seen  in  the  clinical 
phenomena  of  infantile  dystrophies,  of  acromegaly,  and  a  number  of 
related  conditions?  Similar  questions  arise  for  discussion  concerning 
the  thyroid,  the  thymus,  and  the  adrenals.  The  pineal  gland  is  also 
a  chromaffin  formation,  likewise  the  parathyroids,  with  their  problem 
of  regulating  the  calcium  metabolism  not  yet  certainly  disposed  of. 
What  of  the  other  constituents  of  the  body  plasma;  what  keeps  them 
all  in  a  state  of  equilibrium,  so  that  all  types  of  functioning  may  go  on, 
physico-chemical,  vital,  and  psychical? 

This  entire  group  of  questions  cannot  even  be  asked  here.  It 
can  only  be  said  that  in  their  consideration,  one  sees  an  entirely  new 
country  opening  up  which  promises  to  greatly  modify  the  geography 
of  our  present  neurological  schemes.  Sequard  in  1889,  reached  out 
for  immortality  by  his  use  of  testicular  substances.  So  far  as  space 
permits  the  more  essential  interrelationships  and  correlations  will  be 
brought  out  in  the  discussion  of  the  various  diseases.  They  are  at 
present  grouped  under  their  respective  glands.  A  shift  in  the  point  of 
view,  is  apt  to  come  at  almost  any  time. 

The  chief  available  literature  summaries  are  BiedV  Sajous,^  Falta,^ 

1  Internal  Secretions.  ^  The  Internal  Secretions. 

3  Die  Erkrankungen  der  Blutdriisen,  exhaustive  and  suggestive. 


THE  THYREOPATHIES—THYREOSES  133 

Lewandowsky/  Parhon,  and  Goldstein,  and  special  monographs  to  be 
mentioned  under  the  separate  diseases,  such  as  Gushing  on  the 
Pituitary,  Klose  and  Vogt  on  the  Thyvms,  Morel  on  the  Parathyroid, 
Sattler  on  Basedow's  Disease,  etc. 

Among  the  earlier  attempts  at  correlation  of  the  group  of  disorders 
of  the  blood  glands  or  internal  secretory  glands  were  those  of  Glaude 
and  Gougerot,  and  Laignel-Lavastine.  The  latter  proposed  the 
following: 

1.  Uniglandular  syndromes,  with  pluriglandular  lesions,  as  myx- 
edema. 

2.  Pauciglandular  syndromes,  i.  e.,  predominance  of  one  blood 
gland  syndrome  with  changes  occurring  in  others. 

3.  Pluriglandular  insufficiencies  without  predominance  of  one  or 
another.     Dercum's  disease — ovariothyroid. 

4.  Pluriglandular  hyperf unction.     Acromegaly. 

5.  Pluriglandular  balancing,  where  a  hyperfunction  in  one  direction 
is  a  compensation  for  a  lack  in  another.  Thus,  exophthalmic  goitre 
following  ovarian  removal. 

6.  Pluriglandular  disharmony,  as  seen  in  exophthalmic  goitre  with 
myxedema. 

7.  Abortive  types. 

The  French  school  has  carried  these  hypothetical  deductions  to 
great  lengths,  and  have  developed  an  organotherapy  of  startling 
complexity.  As  yet  no  systematic  presentation  is  possible.  Here  we 
purpose  to  discuss  those  disorders  of  the  internal  secretions  with 
pronounced  disturbance  of  the  nervous  system,  begirining  with  those 
best  known. 

We  shall  therefore  take  up:  (1)  the  hypo-,  hyper-,  and  dysthyreoses, 
(2)  the  hypo-,  hyper-,  and  dyspituitarisms,  and  (3)  the  disorders  of 
the  parathyroid,  thymus,  and  adrenals.  Finally,  some  suggestive 
relationships  between  diseases  of  certain  viscera,  liver,  kidneys,  spleen, 
etc.,  and  the  nervous  system. 


THE  THYREOPATHIES ;  THYREOSES. 

Hypothyreoses :  Myxedema. — The  chief  hypothyreoses  are  grouped 
under  the  symbols  myxedema  and  cretinism.  Three  main  types  of  the 
former,  congenital,  idiopathic,  and  operative,  are  described,  while 
sporadic,  endemic,  and  irregular  types  of  cretinism  are  distinguished. 
The  whole  group  may  be  considered  as  one,  i.  e.,  hypo-  and  athyreoses. 
Clinically  the  various  subgroups  have  grown  up  since  Gull,  in  1873, 
first  called  attention  to  myxedema.  They  are  still  in  need  of  clearer 
differentiation  and  description. 

^  Handbuch  der  Neurologic,  vol.  iv,  Special  Neurology,  a  collection  of  valuable 
monographs. 


134  THE  ENDOCRINOPATHIES 

Operative  myxedema  (cachexia  thyreopriva)  has  been  the  best 
studied  type  since  Kocher,  in  1883,  called  attention  to  it,  one  year 
after  Haddon  had  shown  the  relationship  between  myxedema  and  the 
thyroid. 

Symptoms. — The  chief  symptoms  are  present  in  the  skin,  nervous 
system,  thyroid,  circulatory  apparatus,  temperature,  digestive  tract, 
blood,  urine,  bones,  and  general  metabolism. 

Skin. — Here  there  occurs  a  general  gradually  increasing  swelling, 
most  marked  in  the  hands  and  head.  It  seems  edematous  and  yet 
does  not  pit  on  pressure.  The  thickness  of  the  lips  causes  an  eversion 
or  hanging  down  of  the  same.  The  folds  of  the  skin,  especially  of  the 
forehead,  are  more  distinct  than  is  usual.  Irregular,  flattish,  fatty- 
like deposits  are  present  in  different  parts  of  the  body,  often  being 
most  marked  in  the  supraclavicular  region. 

The  hands  are  apt  to  be  clumsy  and  fat,  the  skin  of  the  back  of  the 
hand  being  much  thickened.  The  feet  may  show  similar  changes. 
The  whole  skin  is  whitish  and  dry;  it  scales  readily  and  rarely  shows 
any  perspiration.  Diaphoretic  drugs  even  are  unable  to  bring  about 
any  marked  perspiration,  and  the  mucous  membranes  are  apt  to  be 
dry  and  not  easily  irritated  to  cause  exudates.  Yellowish  pigmenta- 
tion may  occur.  The  hair  breaks  easily  and  is  apt  to  be  badly  and 
sparsely  developed.  The  nails  are  brittle,  develop  slowly,  and  show 
irregular  markings.     The  teeth  also  develop  badly. 

There  is  a  sense  of  tension  in  the  skin,  and  coldness  of  the  extremities 
seems  universal,  and  is  made  much  worse  in  winter,  with  marked 
tendency  to  chapping  and  frost  bites. 

The  nervous  system  shows  a  number  of  defects  varying  with  the 
grade  of  hypothyreosis.  Any  of  the  cranial  nerves  may  show  defective 
development.  The  cerebrospinal  nerves  may  be  deficient.  The 
motility  as  well  as  the  sensibility  is  diminished  both  as  a  result  of 
peripheral  and  central  maldevelopment.  The  changes  in  sensibility 
are  furthermore  augmented  by  the  localized  skin  changes.  The  reflexes 
are  not  markedly  changed.  The  gait  is  usually  wide  based  and 
clumsy  and  dependent  upon  the  mental  substratum.  Defective 
thj^roid  substance  seems  to  hinder  the  regeneration  of  cut  or  injured 
peripheral  nerves. 

Mentally  a  great  variety  of  changes  may  be  observed.  They 
are  usually  in  the  nature  of  defect.  There  is  defect  of  memory,  atten- 
tion is  diminished,  thinking  goes  on  more  slowly,  but  may  be  of 
fair  capacity.  There  is  usually  a  loss  of  initiative,  and  emotional 
dulness  goes  along  with  the  sensory  losses  and  motor  reluctance. 
The  speech  is  apt  to  be  slow  as  are  other  motor  acts.  It  is  monotonous 
and  the  thickened  lips  further  contribute  to  make  it  at  times  unin- 
telligible. The  whole  appearance  of  the  patient  is  one  of  gradually 
advancing  stupidity  which,  if  there  is  no  relief,  goes  on  to  more  pro- 
found defect  states — dementia. 

The  thyroid  itself  is  usually  much  diminished  in  size,  or  not  at  all 


MYXEDEMA  135 

palpable.  Though  palpable  its  active  secretory  substance  is  usually 
defective. 

The  circulatory  apparatus  shows  little  abnormality.  Unlike  the 
hyperthyreoses  there  is  no  contrasting  bradycardia.  The  heart  action 
is  usually  normal — the  larger  vessels  may  be  felt.  Vasoconstrictor 
action  is  prominent  and  is  responsible  for  the  cold  extremities  and 
possibly  some  of  the  dryness  of  the  skin. 

The  cold  sensations  are  not  subjective  alone,  as  th^ere  seems  to  be  a 
fairly  constant  diminution  in  the  bodily  heat,  as  is  also  seen  in 
hypopituitarism.  Digestive  discomforts  from  dry  mouth  and  enlarged 
tongue  are  frequent.  There  is  not  infrequently  diminished  muscular 
tone  and  deficient  secretions  in  the  entire  digestive  apparatus  with 
obstinate  constipation. 

Genital  anomalies  are  frequent,  consisting  of  irregular  or  depressed 
menstruation  or  diminished  potency.  The  organs  themselves — 
testes,  ovaries — may  be  diminished  in  size  and  infantile  defective 
hairy  development  is  present. 

The  blood  shows  fairly  constant  eosinophilia,  and  the  clotting  time 
is  distinctly  increased  and  the  fibrous  content  above  normal. 

The  urine  is  not  characteristically  altered,  save  that  its  quantity  is 
usually  very  low. 

Metabolism  is  slowed  down  in  many  directions.  Oxygen  exchange 
is  reduced,  the  calories  consumed  being  markedly  diminished.  The 
nitrogen  output  is  less,  as  well  as  that  of  the  purin  derivatives.  The 
calcium-magnesium  metabolism  is  not  modified  save  in  the  operative 
individuals,  in  whom  the  parathyroids  are  also  disturbed.  Carbohy- 
drate tolerance  is  high. 

The  bony  system  is  variously  altered.  Here  the  grade  and  com- 
plexity of  change  depends  largely  upon  the  age  of  the  patient  at 
the  onset  of  the  disorder.  The  long  bones  fail  to  develop  normally 
and  those  with  late  ossification  centers  fail  to  undergo  complete 
development.  The  skull  is  apt  to  be  macrocephalic,  but  the  thickness 
of  the  bone  may  diminish  the  interior  capacity  a  great  deal.  The 
fontanelle  in  young  patients  is  apt  to  remain  open.  The  changes  in 
myxedema  are  not  those  seen  in  rachitis  or  chondrostrophia. 

Types. — ^The  idiopathic  form  usually  begins  w^th  changes  in  the 
skin,  and  is  often  accompanied  by  neuralgic  pains.  A  patient  recently 
seen  was  diagnosed  as  a  tabetic.  The  skin  of  the  face  is  often  first 
affected  and  usually  very  gradually  the  extremities  are  implicated. 
The  symptoms  may  all  come  on  within  a  few  weeks,  but  usually  their 
development  occupies  months.  Women  are  much  more  frequently 
observed,  and  usually  about  the  menopause  period,  when  the 
hypothyreosis  may  be  diagnosed  as  a  so-called  "menopause  neurosis." 

Operative  myxedema  is  now  comparatively  rare,  since  the  essential 
relationships  have  been  pointed  out.  The  tetany  symptoms  often  seen 
in  the  earlier  and  badly  operated  cases  were  due  to  the  parathyroid 
removal. 


136 


THE  ENDOCRINOPATHIES 


Congenital  forms,  thyreoaplasias,  occur  in  children  usually  of  nor- 
mal birth,  and  usual  development  up  to  about  the  time  of  weaning 
• — if  not  breast-fed  usually  earlier  (thyroid  in  mother's  milk).  The 
symptoms  then  develop  rapidly  and,  as  a  rule,  are  very  extreme. 
The  irregular,  imperfectly  developed,  cretinoid  pictures  are  not  the 
usual  ones  in  congenital  myxedema;  as  Eppinger  has  remarked  there 
are  few  "half-way"  congenital  thyreoaplasias.  The  female  sex  pre- 
ponderates and  there  are  no  geographical  limitations  as  in  endemic 
cretinism.  Neither  is  there,  as  a  rule,  any  goitrous  family  history  as 
is  often  found  in  cretinism. 

These  little  patients  forget  to  suckle  and  to  swallow.  The  skin 
becomes  folded,  the  nose  broad,  the  eyes  deeply  sunken,  the  nasal 

wings  widely  spread  apart.      They 
are  mouth-breathers,  with  swollen, 
/     ^  not   infrequently    protruding,  cya- 

/  notic   tongues.      Salivary    increase 

is  often  present.  The  hair  is  badly 
developed,  the  face  that  of  an  old 
man.  The  head  grows  in  size,  but 
the  rest  of  the  body  stays  behind 
with  marked  disproportion  in  length 
and  breadth  throughout.  The  epi- 
physes do  not  ossify,  and  the  ossi- 
ficatory  nuclei,  especially  in  the 
hands,  fail  to  develop.  The  fonta- 
nelles  remain  open  perhaps  to  the 
twelfth  or  fifteenth  year,  and  the 
teeth  are  slow  in  appearance. 
The  body  is  apt  to  be  fat  and 
the  abdomen  especially  swollen, 
in  part  from  gas  and  obstinate 
fecal  accumulations.  Umbilical 
hernia  is  frequent.  Other  defects 
are  often  present  in  heart,  palate, 
and  other  structures,  and  they  die  young  with  the  general  mental 
symptoms  in  part  described. 

Diagnosis. — Nephritic  edema  and  other  skin  edemas  must  at  first 
be  ruled  out,  especially  ovarian  pseudoedema  of  the  menopause  and 
rare  forms  of  syphilitic  or  familial  neurotrophic  edema.  Chondro- 
strophia  must  be  separated  from  the  cretinoid  complications.  The 
relations  to  cretinism  are  close.  There  are  differences  in  the  skin 
and  perspiration.  Deaf -mutism  is  rare  in  myxedema,  frequent  in 
cretinism. 

This  whole  problem  is  too  complex  to  discuss  in  a  text-book. 
(See  Eppinger  in  Lewandowsky,  and  Falta,  Erkrankungen  der  Blut- 
driisen.) 

Prognosis  and  Therapy. — See  Cretinism. 


Fig.  46. — Myxedema  showing  failure 
of  ossification  in  epiphyses  of  the  bones 
of  the  hand.     (Siegert.) 


CRETINISM  137 

Cretinism. — This  is  a  broad,  general  term  applied  to  a  combination 
of  physical  and  mental  changes  which,  in  the  young,  result  from  loss 
or  diminution  of  the  thyroid  functions.  Such  a  loss  may  occur  sporad- 
ically, sporadic  cretinism,  from  causes  to  be  enumerated,  where  the 
picture  is  analogous  to  that  seen  in  the  adult  from  removal  of  the 
thyroid,  cachexia  thyreopriva  adultorum,  or  it  may  occur  as  a  localized 
or  endemic  degeneration,  affecting  the  thyroids  of  a  large  number  of 
individuals,  causing  a  hypothyreosis  which  may  show  a  number  of 
tendencies.  The  chief  of  these  are  goitre,  goitrous  heart,  and  endemic 
cretinism.. 

These  three  fairly  well-separated  conditions  may  be  discussed  to 
advantage  under  the  head  of  cretinism.  In  the  first  place  to  call 
cretinism  a  type  of  idiocy  is  misleading.  There  are  numerous  very 
intelligent  cretins.  Cretinism,  as  here  used,  is  solely  a  series  of 
different  conditions  due  to  a  lack  of  development  of  one  or  more 
elements  in  the  body  and  due  to  defect  or  loss  of  the  thyroid  hormones. 

The  historical  chapters  on  cretinism  are  full  of  interest.  The  dis- 
orders w^ere  known  in  early  days.  Pliny  has  left  indubitable  evidence 
of  their  presence  in  early  Roman  times.  Vogt  in  his  admirable 
monograph  in  the  Lewandowsky  Handbuch  der  Neurologic,  tells  of 
Marco  Polo's  descriptions  of  certain  types  he  had  seen  in  his  Asiatic 
travels.  During  the  past  two  centuries  the  disorders  here  included 
under  this  term  have  been  observed  throughout  the  world.  In  cer- 
tain lands  the  disease  is  very  widely  distributed,  certain  mountainous 
districts  of  Switzerland,  Northern  Italy,  etc. — endemic  cretinism — ■ 
while  in  other  regions  it  occurs  rarely — sporadic  cases.  In  the  United 
States  it  is  not  frequent.  It  has  been  observed  in  California,  in  Ver- 
mont, and  such  patients  have  been  seen  in  New  York  State  (Adiron- 
dacks).  In  certain  regions  it  has  been  a  veritable  plague.  Thus  in 
Switzerland  between  the  years  1875-1884,  7  per  cent,  of  the  recruits 
in  the  army  showed  some  form  of  cretinoid  degeneration.  In  ten  years 
2500  men  were  lost  to  the  Swiss  army  from  this  cause  alone.  Certain 
valleys,  especially  those  of  Berne  and  Wallis,  are  over-thickly  popu- 
lated with  individuals  showing  cretinoid  degeneration.  In  the  school 
years  1899-1904,  of  336,000  children  fit  for  school,  15,000  had  one 
or  another  type  of  cretinism.  Similar  conditions  existed  in  Styria, 
Austria,  and  in  certain  Italian  provinces.  F.  Bircher  has  contributed 
an  important  study  to  the  distribution  of  cretinoid  degeneration. 

So  far  as  the  etiology  is  concerned,  it  seems  certain  that  the  conditions 
are  due  to  a  defect  of  the  thyroid  substance — the  thyroid  hormones. 
There  are  certain  limitations  which  must  be  discussed  in  their  respective 
paragraphs. 

Sporadic  Cretinism. — Infantile  Myxedema  of  some  Authors. — The 
clinical  picture  in  an  extreme  case — i.  e.,  fully  developed — in  contrast 
to  the  many  irregular  or  incomplete  forms — is  that  of  a  normally 
born  child  who  about  the  end  of  the  first  or  the  beginning  of  the 
second  year  begins  to  show  the  characteristic  changes  in  development. 


138 


THE  ENDOCRINOPATHIES 


The  little  patients  fall  behind  in  their  normal  bony  development. 

This  is  due  to  a  defect  in  the  development  of  the  long  bones.     The 

epiphyses  fail  to  lay  down  bone  even  after  twenty,  thirty-five  years, 

and  in  twenty-year-old  cretins  the 
anterior  fontanelle  may  still  remain 
open.  There  is  a  proportionate  loss 
in  bone  substance  throughout;  thus 
a  characteristic  dwarfism  results  save 
perhaps  in  the  development  of  the 
skull,  which  develops  larger  in  pro- 
portion to  the  rest  of  the  body.  The 
sphenoid,  however,  fails  to  develop 
and  therefore  gives  the  peculiar  char- 
acteristic nose  to  the  cretin.  The 
bony  defect  is  in  the  nature  of  a 
sclerosis  and  the  bony  tissue  is  un- 
usually hard  which  is  the  reverse  of 
that  seen  in  rachitis. 

Dental  deficiencies  go  hand  in  hand 
with  the  bony  defect.  In  severe 
athyroid  cretins  the  teeth  do  not 
develop  for  a  number  of  years,  and 
the  first  or  milk  teeth  may  persist 
far  beyond  the  normal  period.  Other 
defects  appear  in  a  high  palatine  arch, 
with  large  adenoids  and  tonsils  and 
a  chronic  hypertrophic  rhinitis  causes 
the  child  to  snore  and  sniffle,  often 
with  copious  excretions  from  the 
nose. 

A  fairly  constant  finding  is  that  of 
umbilical  hernia.  The  abdomen  is 
usually  puffy,  the  navel  sunken.  The 
skin  is  myxedematous  in  the  young, 
but  becomes  atrophic  in  later  years, 
the  supraclavicular  and  facial  swelling 
remaining  for  many  years. 

The  facial  habitus  is  characteristic. 
The  hair  line  begins  low.  The  nose 
is  sunken,  the  zygomatic  arches  promi- 
nent, the  eyelids  swollen,  the  face 
puffy,  the  tongue  enlarged  and  often 
protruding  between  the  swollen  lips, 

in  the  mild  cases  giving  one  the  impression  of  a  child  whose  whole 

countenance  is  puffed  up  with  crying. 

There   is   usually   an   enlargement   of  the   liver.     Respiration   is 

unusually  slow  in  the  severe  athyreoses.     The  genital  organs  show 


Fig.  47. —  Cretinism.  Woman, 
aged  thirty-four  years;  mentally, 
seven  years  by  Binet-Simon  test; 
height,  491  inches;  protuberant 
abdomen,  typical  facies,  supraclav- 
icular pads  of  fat. 


CRETINOID  DEGENERATION  139 

marked  changes.  The  labia  are  small,  the  external  not  covering  the 
internal  ones.  The  uterus  and  ovaries  are  usually  small,  and  the 
mammary  glands  are  atrophic  or  hypoplastic.  The  penis  is  apt  to 
be  small,  the  testicles  undescended  and  small.  Genital  hair  and 
that  in  the  arm-pits  is  absent  or  scanty.  In  bo}s  the  pubertal  changes 
in  the  voice  are  lacking. 

Blood  changes  are  present.  The  hemoglobin  is  reduced  and  is 
out  of  proportion  to  the  erythrocytes.  The  leukocytes  are  increased, 
the  polymorphic  neutrophiles  being  markedly  diminished  and  the 
lymphocytes  correspondingly  increased.  Large  numbers  of  granular 
cells  are  observed.  These  changes  appear  as  a  result  of  defective 
thyroid  substance. 

The  metabolism  of  calcium  is  markedly  diminished  (one-third  of 
its  normal  amount  in  the  studies  made  by  Haugardy  and  Langstein) 
and  the  required  calories  are  far  below  that  of  the  normal  child's 
need.  The  assimilation  of  carbohydrates  seems  high,  and  adrenalin 
injections,  without  increased  sugar  intake,  do  not  result  in  a  glycosuria. 
There  is  a  definite  hypothermia.  In  many  cretins  there  is  a  widening 
of  the  sella  turcica.     Hypoplasia  of  the  thymus  is  also  not  infrequent. 

Nervous  system  defects  are  present  with  the  others  and  apparently 
conditioned  by  the  endocrinous  gland  insufficiencies.  These  show 
at  vital  levels  in  defects  of  sensory  and  motor  nerve  structm-es,  and 
at  psychical  levels  in  various  grades  of  stupidity,  mental  weakness 
(moron),  imbecility  or  even  idiocy.  These  words  are  here  used  in 
accordance  with  the  arbitrary  scale  of  the  Binet-Simon  tests. 

Thus  smell  is  at  times  defective.  The  eyesight  poor;  hearing  is 
frequently  distiu-bed,  and  with  it  speech,  so  that  many  patients  are 
deaf  and  dumb.  The  vestibular  function  is  frequently  involved, 
so  that  these  patients  balance  badly,  often  showing  unsteady  gait, 
with  wobbling  of  the  head,  and  nystagmus. 

Some  sporadic  cretins  may  show  little  disease  of  the  nervous  system. 

Cretinoid  Degeneration.— Mention  has  been  made  of  the  widespread 
character  of  this  type  of  degeneration  related  to  defective  or  absent 
thyroid  secretions.  The  statistical  study  of  the  conditions,  particularly 
in  Switzerland,  in  France  and  in  Italy  (Bircher)  has  shown  that  goitre, 
goitrous  heart,  endemic  cretinism,  endemic  deaf  and  dumbness,  and 
endemic  feeble-mi ndedness  are  closely  allied.  The  cretins  are  almost 
all  goitrous,  or  nearly  always  have  goitrous  parents.  Exophthalmic 
goitre  (hyperfunction)  is  rare  with  cretinism,  but  very  frequent  with 
goitrous  heart  conditions. 

The  causes  of  the  hypothyroidism  are  not  definitely  settled  but 
there  seems  to  be  a  constant  relation  between  it  and  certain  elements 
in  the  water  supply,  and  goitrous  springs  are  known.  Just  what 
the  noxious  element  may  be  is  still  conjectural  but  it  apparently 
is  related  to  mineral  constituents  found  in  certain  geological  formations, 
notably  in  the  Trias  and  Tertiary.  The  disease  is  absent  in  regions 
fed  from  waters  of  crystalline  formation.    Through  Bircher's  sugges- 


140  THE  ENDOCRINOPATHIES 

tion  of  supplying  a  goitrous  region  in  Rupperwill  from  Jura  water 
coming  from  an  adjacent  valley,  the  disease  disappeared.  Similar 
results  followed  in  the  town  of  Asp.  Animals  may  be  made  goitrous 
from  drinking  water  from  certain  springs.  They  also  develop  goitrous 
hearts,  and  are  delayed  in  their  development.  The  thyroid  shows 
degenerative  changes.  The  agent  passes  through  a  Berkefeld  filter, 
but  is  modified  and  made  non-active  by  being  heated  to  70°  C.  It 
does  not  dialyze  and  is  thought  not  to  be  an  organized  plant  or 
animal  substance,  but  is  of  a  colloidal  nature.  An  hypothesis  which 
had  the  authority  of  Bircher  behind  it  was  that  the  disease  was  of  an 
infectious  nature. 

Goitre. — Here  Falta  describes  those  enlarged  hyperplastic,  non- 
inflammatory thyroid  formations,  with  degenerative  changes  in  the 
struma.  The  hyperplasia  invades  the  parenchyma  and  the  vessels. 
Notwithstanding  histological  hyperplasia  there  is  physiological  dim- 
inution in  function.  Functionating  parenchyma,  however,  does  not 
exist.  Histologically  one  finds  parenchymatous,  vascular,  or  fibrous 
hyperplasias,  with  circumscribed  or  diffuse  goitres.  A  relationship 
between  goitre  and  uterine  myomata  exists,  and  with  a  diminution 
of  one  there  is  a  decrease  of  the  other  at  the  menopause. 

Goitre  Heart. — See  Exophthalmic  Goitre. 

Endemic  Cretinism. — Here  there  is  a  richer  and  much  more  variable 
picture  than  obtains  for  sporadic  cretinism.  Whereas  a  typical 
habitus  is  described,  there  are  many  anomalies  and  variations.  The 
head  is  usually  broad,  but  may  be  small  and  flat,  instead  of  large 
and  broad,  at  times  very  large.  The  nose  is  usually  wide  spreading 
and  flat,  the  eyes  wide  apart.  The  neck  is  short  and  thick,  the  features 
swollen,  the  early  impression,  especially  of  the  prognathism,  one  of 
moroseness  or  stolidity.  The  bones  are  shortened,  various  anomalies 
as  scoliosis,  ankyloses,  etc.,  being  present.  Great  variation  in  dwarfism 
is  observed.  Certain  cretins  are  under  three  feet  six  inches,  but  full 
cretins  have  been  observed  of  seven  feet  in  height.  As  a  rule  they  die 
young,  but  Kocher  reports  cretins  seventy  and  even  one  hundred 
years  of  age. 

The  general  coordination  of  these  patients  is  poor.  They  are 
usually  short,  clumsy,  inelastic  with  badly  developed  musculature. 
The  skin  is  loose,  lax,  anemic,  marked  with  folds  and  wrinkles,  giving 
a  peculiar  appearance  of  old  age.  The  lips  are  swollen,  the  tongue 
enlarged,  and  not  infrequently  protruding.  The  breasts  are  flat 
or  badly  developed,  the  abdomen  flat  or  pendulous.  Short,  stumpy 
fingers  and  toes  give  an  ugly  appearance  to  the  extremities  and  con- 
tribute to  clumsiness.  The  entire  activity  is  apt  to  be  heavy  and 
awkward,  although  a  few  athletes  and  acrobats  may  be  found  among 
cretins. 

The  changes  in  the  bones  have  been  mentioned  in  the  paragraphs 
on  sporadic  cretinism.  Here,  however,  the  variations  are  more 
marked  and  Weygandt's  study  of  Virchow's  material  shows  that  many 


ENDEMIC  CRETINISM  141 

bony  anomalies  exist  among  cretins  not  mentioned  in  Virchow's 
classic  which  has  remained  a  standard  for  writers  for  many  years. 

The  skin  has  a  peculiar  cachexia.  It  is  swollen  and  flabby,  whitish 
or  yellowish,  folded  and  soggy.  The  general  appearance  of  old  age 
is  striking.  The  hair  and  nails  are  badly  developed,  both  breaking 
easily.  Thick,  underlying,  fatty  masses  are  unevenly  distributed, 
usually  in  the  neck,  back,  upper  chest  regions,  occasionally^  over  the 
hands.  Variable  states  of  tension  occur  in  these  fatty  masses;  at 
times  they  are  hard,  again  like  empty  sacks.  The  mucous  membranes 
are  also  pale  and  gray,  often  folded  but  look  different  from  a  typical 
anemia. 

The  sexual  organ  changes  have  been  touched  upon  in  the  description 
of  sporadic  cretinism.  They  are  characteristically  infantile.  Men- 
struation is  scanty,  wanting,  or  develops  very  late.  Fecundation 
may  take  place,  but  the  results  are  either  miscarriages,  dead  children, 
monsters,  etc.  The  secondary  sexual  characters  are  all  delayed 
in  their  development. 

The  majority  (63  per  cent.,  Ewald)  of  cretins  show  a  swollen 
thyroid,  but  it  is  not  an  overfunctioning  one,  nor  do  they  all  show 
athyreosis,  or  hypothyrosis.  Schonemann  has  reported  the  findings 
of  strumous  changes  in  the  glandular  portion  of  the  hypophysis. 
In  112  autopsies  on  endemic  cretins  he  found  a  normal  hypophysis 
in  only  twenty-seven  instances.  These  individuals  had  no  goitre. 
He  states  that  in  individuals  with  struma  of  the  thyroid  almost  invari- 
able there  was  an  increase  in  the  size  of  the  hypophysis,  and  chiefly 
consisting  of  increase  in  the  connective  tissue,  also  the  chromaffin 
cells,  struma  of  the  vessels,  hyaline  degeneration  and  swelling  of  the 
cell  strands  and  finally  goitres  with  colloid  formation.  It  is  highly 
probable  therefore  that  the  goitre  poison  works  deleteriously  upon  the 
hypophysis  (Falta).     The  parathyroids  show  no  changes. 

Most  of  the  internal  organs  show  reduction  in  activity.  Digestion 
is  usually  slow,  constipation  is  marked.  The  metabolism  is  modified 
as  already  indicated.  The  urinary  secretions  are  apt  to  be  diminished, 
and  of  high  specific  gravity. 

Mentally  cretins  show  marked  variability.  A  few  are  practically 
normal,  but  most  show  a  characteristic  combination  of  mental  traits, 
which  is  in  marked  contrast  with  many  other  defective  mental  states. 
(See  chapters  on  Feeble-mindedness.) 

As  noted,  the  great  majority  suffer  from  impairment  of  the  chief 
sensory  tracts.  Hearing  seems  to  suffer  most.  The  defect  in  hearing 
is  associated  with  speech  defects.  Taste  and  smell  are  also  defective. 
They  take  little  interest  in  their  food  or  drink.  The  feeble-mindedness 
is  accompanied  by  great  slowness  of  all  reactions,  with  marked  retar- 
dation of  motion,  with  apathy,  and  indolence.  This  indolence  is  a 
marked  feature.  Many  cretins  will  lie  in  the  sun  all  day  long,  and  in 
the  hospital  or  other  institution  will  sit  around  and  do  nothing  for 
weeks  or  months.     In  the  milder  grades  there  is  often  great  shyness 


142  THE  ENDOCRINOPATHIES 

which  makes  them  unapproachable  and  serves  to  make  them  appear 
more  feeble-minded  than  they  really  are.  It  is  with  the  greatest 
difficulty  that  they  can  be  trained  to  the  simplest  of  performances. 
With  many,  in  spite  of  the  marked  general  stolidity  of  their  average 
mood,  they  may  show  great  excitement  and  emotional  outbreaks. 

The  sense  of  sight  is  frequently  diminished.  It  is  highly  probable 
that  the  receptors  and  conduction  paths  are  less  involved  than  the 
perception  areas  in  this  diminution  in  sensory  intake.  The  hearing 
seems  to  be  affected  both  as  to  its  receptors  and  to  the  conduction 
paths.  Pain,  touch  and  thermal  sensibilities  are  all  dulled.  Motility 
is  extremely  retarded.  The  reflexes  are  active  (50  per  cent.).  The 
field  of  vision  is  reduced  in  many,  although  the  fundus  is  usually 
normal  (Hitschmann). 

Aberrant  and  abortive  types  are  to  be  expected.  In  the  former 
one  may  find  patients  with  striking  development  of  one  or  more 
features,  in  the  latter  a  very  great  shading  off  to  almost  normal  states, 
i.  e.,  endemic  goitre  with  mild  mental  signs. 

Endemic  Deaf-mutism. — This  combination  is  extremely  frequent 
where  endemic  cretinism  is  present  (29  per  cent.,  Scholz).  It  may 
constitute  one  of  the  aberrant  types  just  mentioned  with  striking 
development  of  single  features,  or  it  may  be  associated  with  all 
the  grades  of  a  complete  cretin  picture.  According  to  the  studies 
of  Kocher,  the  loss  of  hearing  is  due  to  a  bony  defect  which 
has  destroyed  the  possibility  of  normal  cochlear  development. 
Bad  hearing  is  reported  at  32  per  cent,  among  cretins  in  Scholz's 
investigations. 

The  changes  found  in  the  brain  which  may  account  for  the  feeble- 
mindedness have  been  variable.  Meningeal  inflammation  and  mild 
grades  of  hydrocephalus  have  been  found  by  Scholz  and  Zingerle. 
The  brain  is  often  asymmetrical,  small,  or  single  lobes  are  diminished 
in  size.  Often  the  brain's  development  is  arrested  at  an  infantile 
stage,  the  pallium  or  the  ganglia  being  involved  alone  or  together. 
The  cerebellum  is  often  imperfectly  developed,  which  fact  stands  in 
correlation  with  the  marked  incoordination  and  possibly  in  relation 
with  defective  labyrinthine  development. 

The  ear  difficulties  are  numerous.  Peripheral,  conducting  and  central 
mechanisms  are  found  to  be  at  fault,  but  at  all  events  seem  secon- 
dary to  the  developmental  anomalies  induced  by  the  action  of  the 
poisonous  substance  on  the  thyroid.  The  speech  defects  usually  go 
hand  in  hand  with  those  of  hearing,  but  this  is  not  universal.  The 
cortical  developmental  defect  is  sufficiently  explanatory  for  most  of 
the  cases. 

Therapy.  —  Many  contradictions  may  be  found  in  the  literature 
concerning  the  use  of  thyroid  substance  in  various  types  of  myxedema 
and  cretinoid  degeneration.  This  is  to  be  expected  since  so  many 
observers  use  their  diagnostic  terms  so  lightly.  Age  differences  are 
not  recorded — stage  and  intensity  of  the  disease^ — and  grade  of  defect 


TREATMENT  OF   THYREOSES  143 

are  overlooked,  and  hence  no  uniform  basis  for  comparisons  have 
existed. 

Among  the  best  reported  results  are  those  of  v.  Wagner  who  obtained 
a  diminution  in  the  myxedematous  swelling  of  the  skin;  the  genitals 
developed  rapidly,  the  tongue  diminished  in  size,  there  was  loss  of  the 
umbilical  hernia,  development  of  new  hair,  dentition  was  hastened, 
closure  of  the  fontanelles  occurred,  and  there  was  an  increase  in  bony 
development.  The  psyche  was  less  hopefully  modified,  but  there  was 
a  diminution  in  the  apathy,  and  slight  increase  in  the  intellectual 
capacity  was  obtained. 

Early  therapy  is  naturally  Ihe  main  feature.  According  to  v.  Wag- 
ner, small  doses  of  iodine  in  addition  seemed  to  stimulate  the  thyroid 
activities  still  further.  Magnus  Levy,  v.  Eysselt,  and  others  also 
report  excellent  results,  complete  cure  resulting  in  some  patients 
still  in  their  teens. 

A  widespread  state  experiment  carried  out  by  v.  Kutscheras  in 
Styria  treated  1011  cretins.  A  large  number  were  neglected  by  the 
parents  i.  e.,  treatment  was  not  kept  up.  In  2.4  per  cent,  the  thyroid 
tablets  could  not  be  well  borne.  All  idiots  and  severe  grades  of 
deafness  and  dumbness  were  left  alone.  Of  440  of  the  cases  10.2 
per  cent,  showed  slight  increase  in  bony  development,  4  per  cent, 
showed  definite  change,  85  per  cent,  showed  an  increase  well  above  the 
average.  The  increase  in  bony  growth  was  marked  with  the  younger 
individuals,  but  also  persisted  into  the  third  decade. 

A  careful  revision  of  677  cases  showed  42.8  per  cent,  marked  im- 
provement, 48  per  cent,  some  definite  improvement,  8.6  per  cent,  no 
improvement. 

Scholz's  experiences  with  100  cretins  in  an  institution  were  dis- 
appointing. He  used  as  many  as  eight  tablets  a  day.  V.  Wagner 
claims  the  doses  were  too  extreme,  hence  the  bad  results.  Emaciation, 
weakness,  loss  of  appetite,  vomiting  and  diarrhea,  and  other  symptoms 
of  hyperthyroidism  developed,  v.  Wagner  recommends  the  use  of 
only  one-half  or  one  tablet  (thyroidinum  siccum,  Merck  =  gram  0.1); 
0.4  gram  corresponds  to  the  activity  of  an  entire  gland.  (Burroughs 
Welcome  Co.,  0.1-0.3  gram  =  gr.  iss-iij.) 

lodothyrine  has  also  been  utilized.  One  gram  contains  three  milli- 
grammes of  active  substance  representing  the  iodine  content  of  one 
gram  of  fresh  sheep's  thyroid.  It  would  seem  that  the  iodine  content 
is  not  the  only  factor  in  the  activity  of  the  thyroid  substance  and  it  is 
not  as  yet  definitely  demonstrated  what  the  combination  is  that  is 
effective.  Surgical  implanting  of  the  thyroid  gland  itself  would  be 
the  ideal  therapy,  and  experiments  directed  to  this  end  have  been 
carried  out  since  1889  when  Bircher  was  one  of  the  first  to  attempt 
it.  The  gland  has  been  implanted  in  different  portions  of  the  body — 
the  neck,  under  the  breast,  in  the  spleen,  even  in  the  bony  substance. 
As  a  rule,  however,  the  implantation  has  not  been  as  successful  as 
was  hoped,  the  gland  itself  undergoing  retrograde  changes.     Further- 


144  .     THE  ENDOCRINOPATHIES 

more  it  would  appear  from  the  studies  by  Enderlen  and  Borst  that 
thyroids  from  other  animals  possibly  are  not  the  best  things  to  use  as 
the  biochemical  composition  of  the  human  and  animal  types  varies 
so  widely  as  to  render  degeneration  of  the  implanted  gland  likely. 
Implantation  of  human  glands  has  not  been  successfully  brought  about 
as  yet,  but  with  the  newer  work  on  organ  transplantation  as  inaugu- 
rated through  the  researches  of  Carrel  it  would  seem  that  this  technical 
difficulty  might  be  overcome  in  the  very  near  future. 

One  is  compelled,  therefore,  to  resort  in  most  cases  to  the  dried 
or  liquid  preparations  of  the  thyroid  itself  or  to  such  biochemical 
products  as  laboratory  research  has  provided  through  the  utilization 
of  the  glandular  substance  itself  or  that  portion  of  it  which  presents 
its  chief  hormone  activity. 

The  general  results  of  thyroid  medication  in  typical  cases  is  fairly 
constant.  Especially  is  it  of  value  in  the  aberrant  and  minor  forms 
of  the  disease  of  which  one  of  the  most  chronic  of  symptoms  is  the 
persistent  anemia.  This  may  be  in  part  overcome  by  the  simultaneous 
use  of  small  doses  of  arsenic,  which  have  been  recommended  by  a 
number  of  experimenters.  Alcohol  and  morphine  work  disadvantage- 
ously,  and  should  be  carefully  avoided.  The  use  of  small  doses  of 
sodium  bicarbonate  and  bismuth  work  advantageously  in  diarrheal 
states. 

Thyroid  medication  for  the  sporadic  cases  varies  somewhat  from 
its  use  in  the  endemic  cases.  In  the  sporadic  cases  of  the  light  or 
mild  type  the  action  is  quite  similar  to  that  seen  in  the  endemic  ones, 
but  as  a  rule,  sporadic  cases  by  reason  of  their  longer  involvement 
and  the  less  rapid  development  of  the  symptoms,  their  more  hidden 
or  obscure  nature  with  their  great  mixture  of  syndromes  make  such 
cases  less  responsive  to  the  therapy.  Nevertheless,  many  of  them 
respond  very  kindly  to  it,  the  same  dosage  being  utilized. 

Hyperthyreoses :  Exophthalmic  Goitre. — Graves  described  the  con- 
dition in  1835,  Basedow  in  1840.  Mobius  in  1886  insisted  on  the 
relation  of  the  disorder  to  changes  in  the  thyroid  gland. 

Exophthalmic  goitre  is  a  disorder  due  to  a  modification  of  the 
activity  of  thyroid  gland  substance  which  in  turn  leads  to  an  increased 
activity  of  the  vegetative  nervous  system  with  a  series  of  cardiovascular 
signs,  tachycardia,  exophthalmos,  tremor  and  increased  metabolic 
activity. 

The  disorder  is  widespread  and  presents  many  variations.  Indeed 
abortive  forms  are  among  the  commonest  of  the  manifestations  of 
hyperthyroidism.  Women  much  more  often  than  men  show  this 
particular  type  of  disturbance.  In  Sattler's  great  monograph  3200 
of  3800  cases  reported  were  females.     Hereditary  cases  are  known. 

Symptoms. — The  chief  symptoms  are  found  in  the  thyroid,  eye, 
heart  and  bloodvessels,  skin,  and  muscles.  Psychical,  gastro-intestinal, 
respiratory,  genital,  and  metabolic  changes  occur  as  well. 

An  enlarged  thyroid  is  fairly  constant  although  occasionally  absent. 


EXOPHTHALMIC  GOITRE 


145 


It  is  soft  and  elastic,  rich  in  new  bloodvessels,  pulsates,  and  varies  in 
volume  (often  very  rapidly)  fairly  commensurate  with  the  intensity  of 
the  symptoms.  Auscultation  of  the  enlarged  thyroid  often  gives  a 
marked  bruit. 

The  heart  action  is  rapid  (tachycardia),  and  the  pulse  is  very 
variable,  reacting  excessively,  particularly  to  psychical  influences.  The 
heart  sounds  are  increased  in  force,  the  beating  being  felt  in  the  neck, 
and  the  whole  chest  wall  is  at  times  moved  by  the  cardiac  tumult. 
The  blood-pressure  is  rarely  raised  and  the  radial  and  other  vessels 
show  marked  hypotonus  with  reddening  of  the  face,  ears,  and  finger 
extremities. 


Fig.  48. — Exophthalmic  goitre,  show- 
ing marked  exophthalmos  and  enlarged 
thyroid.  (Courtesy  of  Dr.  George  W. 
Crile.) 


Fig.  49. — Same  patient  four  months 
after  operation  (extirpation).  Greatly 
diminished  exophthalmos  and  change  of 
facial  expression.  (Courtesy  of  Dr. 
George  W.  Crile.) 


The  eye  symptoms  consist  of  a  marked  and  variable  protrusion  of 
the  eyeball,  with  widened  orbital  fissure,  sometimes  greater  on  one 
side  than  the  other,  and  felt  as  a  disagreeable  pressure  and  tension  by 
the  patient.  The  eyelids  are  at  times  swollen,  and  the  upper,  and 
possibly  the  lower  lids  largely  retracted  (Dalrymple,  Stellwag) 
independently  of  and  often  preceding  the  protrusion  of  the  eyeballs. 
The  upper  lid  also  does  not  move  synchronously  with  the  lowering  or 
the  raising  of  the  eyeball  (von  Graefe's  symptom),  following  more 
slowly  or  receding  more  rapidly  (spasticity)  than  the  moving  eyeball, 
in  the  presence  or  absence  of  protrusion.  Both  signs  may  be  unequally 
present.  The  relative  infrequency  of  winking  (Stellwag)  is  a  frequent 
10 


146 


THE  ENDOCRINOPATHIES 


symptom.     Eppinger  shows  in  tabular  form  the  relative  frequency 
of  these  ocular  phenomena. 


Symptom. 


Protrusion,  wide,  Graefe 
Protrusion,  Graefe 
Protrusion        .      .       .       . 
No  eye  symptoms 
Wide,  protrusion  . 
Graefe,  wide     .       .       .       . 

Wide 

Graefe 


Number  of  cases 


Wilbrand  and 
Sanger. 
Per  cent. 

Sattler. 
Per  cent. 

23.0 

37.3 

36.0 

19.8 

10.4 

17.6 

15.3 

13.3 

2.2        i 

2.6 

5.4 

5.1 

1.1 

7.6 

3.3 

39.0 

91.0 

Eppinger. 
Per  cent. 


35.6 
16.4 
19.2 

4.8 
6.7 
9.6 
3.8 
3.8 

104.0 


Lowi's  symptom  (dilatation  from  adrenalin)  is  frequent.  Occasion- 
ally mydriasis  is  present,  less  frequently  miosis.  Anisocoria,  or  stiff 
pupils,  may  be  observed  as  well  as  loss  of  the  accommodation  reflexes. 
Optic  atrophy  is  an  infrequent  occurrence.  Increased  tear  secretion  is 
often  observed  early,  dryness  late  in  the  disease.  Mechanical  compli- 
cations— pus,  ulcerations — are  met  with. 

Insufficiency  of  convergence,  without  double  vision  (Mobius  sign),, 
is  frequent,  and  is  independent  of  protrusion. 

Cardiovascular  symptoms  are  among  the  most  constant  and  early 
signs  of  exophthalmic  goitre,  and  are  due  to  the  action  of  the  thyroid 
secretion.  Tachycardia  (occasionally  paroxysmal)  is  the  most  promi- 
nent single  sign.  The  pulse  may  beat  from  100  to  160  times  a  minute 
— even  a  200  beat  has  been  recorded.  Undoubted  cases  may  show  no 
rise  above  100,  and  great  variability  is  the  rule,  especially  in  response 
to  psychical  stimuli.  During  sleep,  and  also  on  lying  down,  the  pulse 
frequency  drops. 

Angina-like  attacks,  with  hyperalgesia  in  the  left  ulnaris  region 
are  not  infrequent,  and  in  most  patients  the  feeling  of  distress  and 
anxiety  over  an  increased  sense  of  heart  oppression  (apart  from  the 
tachycardia)  is  one  of  the  most  annoying  symptoms.  Cardiac  dilata- 
tion, with  later  hypertrophy,  without  valvular  defect  may  or  may  not 
(50  per  cent.)  accompany  the  disease,  and  disappear  at  its  termination. 
On  auscultation  the  first  sound  is  usually  accentuated,  and  systolic 
murmurs  at  the  base  are  frequent.  Valvular  insufficiencies  occur 
under  special  circumstances,  and  are  often  of  serious  consideration 
in  operative  cases. 

Strong  pulsation  of  the  carotids  is  frequent,  and  though  the  large 
vessels  are  often  prominent,  and  apparently  arteriosclerotic,  the 
walls  are  usually  soft  and  yielding.  Vasomotor  instability  is  frequent. 
Marked  reddening  alternates  with  paleness.     Irregular  erythemata 


EXOPHTHALMIC  GOITRE  147 

also  are  not  infrequent  and  many  patients  complain  of  surface  heat, 
seek  cold  places  and  light  clothing,  even  in  winter,  and  yet  show  no 
temperature  anomalies.  Dermographism  is  also  a  frequent  vasomotor 
phenomenon,  as  well  as  not  infrequent  nasal  bleeding.  Rarer  urticarlse, 
irregular  circumscribed  edemas,  pruritis,  etc.,  are  to  be  expected. 

Skin  symptoms  are  frequent.  Increased  perspiration  is  not  rare 
and  the  skin  is  always  moist  with  some  patients,  possibly  one-sided, 
or  in  isolated  (head)  areas  or  smaller  spots. 

The  electrical  resistance  of  the  skin  (Veraguth-Vigoureux)  is  dimin- 
ished as  a  result  of  this  increased  secretory  activity.  Gooseflesh 
develops  readily  with  these  patients,  and  changing  pigmentary  anom- 
alies, chiefly  chloasma  spots,  are  present  on  the  skin,  not  on  mucous 
membranes,  more  especially  on  the  eyelids,  neck,  nipples,  armpits, 
and  genitals.  In  many  patients  (23  per  cent. — Sattler),  there  is  a 
tendency  for  the  hair  to  fall  out  (sometimes  unilaterally)  with  the 
development  of  the  disease,  and  usually  there  is  renewed  growth 
of  the  hair  with  improvement. 

Tremor  is  an  early  and  frequent  symptom,  and  may  involve  the 
entire  body.  It  is  extremely  fine,  varies  from  seven  to  forty  vibra- 
tions per  second,  and  occasionally  is  intermingled  with  wider  irregular 
choreic-like  movements.  Psychical  rather  than  physical  stimuli 
increases  the  tremor  greatly;  lying  down  tends  to  diminish  it.  It 
is  more  apparent  in  the  upper  than  in  the  lower  extremities  and 
is  marked  in  the  eyelids  (Rosenbach),  in  the  tongue,  and  when  in  the 
vocal  cords  produces  a  peculiar  staccato  breathing  (Minor). 

Digestive  disturbances  are  frequent.  Dry  mouth  may  alternate 
with  excessive  salivation.  There  is  a  marked  tendency  to  chronic 
alvine  discharges  (30  per  cent.)  and  to  vomiting  without  anorexia 
(15  per  cent.).  Both  occur  in  paroxysms,  somewhat  resembling 
tabetic  crises.  Hour-glass  contraction  of  the  stomach  may  be  demon- 
strated by  the  .r-rays.  Both  the  vomiting  and  diarrhea  are  obstinate, 
are  accompanied  by  mucus  or  colloid,  at  times  bloody  material  and 
the  diarrhea  may  occur  as  often  as  four  or  five  times  a  day.  Fatty 
stools  without  diarrhea  may  occur.  With  both  diarrhea  and  vomiting 
the  patients  are  in  grave  danger.  Obstipation  of  spastic  type  may  also 
occur.  In  many  patients  there  are  enlarged  lymphatics,  tonsils, 
tongue  follicles,  thymus,  and  lymphatics  of  the  intestines. 

Respiratory  symptoms,  dyspneic  in  character  are  usual.  Normal 
breathing  is  frequently  irregular  in  depth  and  rhythm,  and  seems 
strained.  The  swollen  gland  may  cause  relative  stenosis.  Asthmatic 
tendencies  are  present,  and  the  general  sense  of  air  hunger  is  striking, 
with  nervous  pseudohysterical  coughing. 

Menstrual  irregularities  are  common.  The  flow  is  usually  small 
in  amount  and  infrequent  in  occurrence,  with  occasionally  the  direct 
reverse  condition.  Thinning  of  the  breasts,  and  other  atrophies 
(testicles)  have  been  recorded,  and  seem  to  be  coordinated  with 
thymus  anomalies. 


148 


THE  ENDOCRINOPATHIES 


Metabolic  anomalies  are  characteristic.  The  patients  become 
markedly  emaciated  and  get  very  weak.  This  is  related  to  a  definite 
nitrogen  loss,  and  also  to  a  marked  overoxidation  of  carbohydrates 
and  fats  (see  fatty  stools).  This  sudden  loss  of  flesh  and  strength 
may  come  on  in  attacks,  and  then  a  distinct  improvement  takes 
place.  Eppinger  speaks  of  these  patients  as  individuals  who,  not 
doing  any  work,  need  all  the  calories  of  a  hard  working  individual. 
Thus  in  mild  cases  an  increase  in  calories  keeps  the  patient  at  a  normal 
weight.  The  increased  oxidation  also  shows  in  a  mild  hyperthermia. 
Alimentary  glycosuria  is  frequent,  and  gradually  disappears  on  re- 


FiG.  50. 


-Exophthalmic  goitre. 
(Hammond.) 


Fig.  51. 


-Exophthalmic  goitre. 
(Hammond.) 


CO  very;  true  diabetes  is  an  infrequent  complication.  Polyuria  is 
frequent  (13  per  cent.,  Sattler),  less  so  albuminuria  (11  per  cent., 
Sattler). 

The  hlood  shows  anemic  changes  frequently  at  the  very  outset. 
The  number  of  red  cells  is  not  markedly  lowered  as  a  rule  save  in 
those  patients  with  marked  cachexia.  The  leukocyte  count  is  usually 
low;  the  percentage  of  cells  is  altered.  Lymphocytosis  is  marked 
(60  per  cent.) .  The  polynuclear  neutrophiles  are  markedly  diminished. 
The  eosinophile  cells  are  increased  (8-20  per  cent.).  The  large  mono- 
nuclears are  normal.  Thyroidectomy  changes  the  entire  blood  picture 
more  towards  normal,  as  does  also  ligation  of  the  thyroid  arteries 
and  according  to  Kocher  the  blood  picture  is  a  valuable  prognostic 


EXOPHTHALMIC  GOITRE  '  149 

index.  The  coagulation  time  is  increased.  In  the  young,  hyper- 
thyreosis  leads  to  increased  growth  of  the  bones  and  young  exophthal- 
mic patients  are  apt  to  l)e  very  large. 

The  mental  symptomatology  of  hyperthyreosis  is  of  great  importance, 
since  from  the  studies  of  Parhon  and  others  it  seems  possible  that  like 
others  of  the  symptoms  the  mental  signs  may  develop  almost  exclu- 
sivel}'.  In  many  cases  the  psychical  signs  are  mild.  The  tendency  is  to 
both  psychomotor  and  emotional  irritability.  Moodiness  and  sudden 
changes  are  frequent.  In  marked  stages  distinctly  manic  phases  may 
develop;  again  acute  and  deep  depressions  (often  suicidal)  take  their 
place.  Thus  the  picture  approaches  very  closely  at  times  to  the  type 
of  Kraepelin's  mixed  manic-depressives,  or  the  more  typical  circular 
forms  of  this  psychosis.  Toxic  epiphenomena  may  take  place  with 
ideas  of  reference,  of  persecution,  even  hallucinations,  principally  of 
sight.     The  general  picture  of  an  acute  delirium  is  a  grave  sign. 

The  analj'^sis  of  the  psychical  pictures  in  exophthalmic  goitre  is 
far  from  complete.  Sattler  advocates  a  catholic  attitude,  saying 
there  is  no  one  psychotic  pictiire,  a  typical  hyperthyroid  psychosis, 
but  the  present  tendency  is  to  ally  the  mental  phenomena  of  the 
hyperthyreoses  with  the  manic-depressive  group. 

General  Etiology  and  Pathology. — General  considerations  relative  to 
the  athyreoses,  dysthyreoses,  and  hyperthyreoses  as  seen  in  the  clinical 
pictures  of  congenital  and  acquired  hypofunctioning  as  in  cretinism 
and  myxedema,  and  in  hyperfunctioning  as  seen  in  exophthalmic 
goitre  point  to  paralytic  and  irritative  phenomena  of  the  sympathetic 
and  parasympathetic  (autonomic)  nervous  systems. 

The  blood  changes  in  exophthalmic  goitre  and  in  myxedema  are 
similar,  i.  e.,  there  is  a  relative  lymphocytosis  with  diminution  of  the 
neutrophile  leukocytes.  In  exophthalmic  goitre  the  coagulation  time 
is  increased,  in  myxedema  diminished.  In  exophthalmic  goitre  the 
sympathetic  irritation  explains  the  exophthalmos,  tachycardia,  loss 
of  weight,  and  the  alimentary  glycosuria.  Autonomic  irritability 
causes  the  von  Graefe,  the  lymphocytosis,  the  diarrhea,  the  increased 
secretions.  The  influence  of  the  thyroids  on  the  carbohydrate  metabol- 
ism, as  seen  in  the  rapid  emaciation  and  alimentary  glycosuria,  possibly 
acts  through  the  pancreatic  retardation  or  through  a  relative  increase 
in  adrenalin  action.  That  the  thymus  is  involved  in  the  blood  picture 
formation  seems  certain. 

Thus  one  comes  to  a  combined  neurochemical  theory  in  that 
exophthalmic  goitre  is  dependent  upon  hyperactivity  of  the  thyroid 
secretions,  which  increased  secretions  act  through  the  visceral  or 
vegetative  nervous  system.  Both  autonomic  and  sympathetic 
systems  are  thus  in  a  state  of  hyperexcitability — a  condition  the 
anatomical  foundations  for  which  are  found  in  a  certain  type  of 
individual  termed  vagotonic  by  Eppinger. 

The  detoxication  hypothesis  of  Blum,  Ostwald,  Kocher,  Klose  and 
others,  in  which  a   dysthvreosis   is   assumed   and   that   the   disease 


150  THE  ENDOCRINOPATHIES 

is  really  a  type  of  iodine  poisoning  from  insuflficient  detoxication  is 
ingenious. 

The  full  etiology  is  still  very  dark.  Many  apparently  healthy 
individuals  suddenly  develop  the  disorder  following  a  shock.  This 
shock  frequently  involves  the  complex  of  the  fear  of  death,  loss  of 
money.  Minute  analyses  from  the  psychoanalytic  school  are  not  yet 
available  to  permit  generalization,  but  the  psychical  import  of  shock 
is  undoubted. 

An  infectious  disease  type  is  also  recognized — acute  thyroiditis, 
and  also  a  type  of  iodine  intoxication  in  individuals  who  have  taken 
potassium  or  other  iodides. 

The  changes  in  the  gland  itself  are  of  little  moment  for  the  inter- 
pretation of  the  disorders.  A  great  variety  of  variations  from  the 
normal  have  been  described,  chiefly  of  hyperplastic  type.  Apparently 
perfectly  normal  glands  are  at  times  associated  with  severe  types 
of  the  disease.  The  gland  is  usually  enlarged,  elastic,  the  vessels 
dilated,  and  new  proliferating  bloodvessels  are  found.  Kocher  has 
described  the  goitrous  type  as  parenchymatous  hyperplastic  struma, 
poor  in  colloid  and  in  iodine;  other  changes  are  largely  due  to 
complications. 

Forms  and  Diagnosis. — If  the  classical  triad,  exophthalmos,  tachy- 
cardia, and  goitre  be  present,  there  is  little  question  as  to  diagnosis, 
but  still  all  may  be  absent  and  yet  the  patient  be  suffering  from  severe 
hyperthyroidism.  Hence  great  variability  may  be  expected.  Eppinger 
and  Hess  distinguish  two  chief  groups  according  to  the  predominance 
of  the  sympathetic  or  autonomic  irritative  phenomena.  These  de- 
serve more  detailed  study.  The  autonomic  group  in  particular  is 
often  overlooked.  These  show  von  Graefe's  sign,  diarrhea,  lympho- 
cytosis and  increased  perspiration  and  marked  anxiety.  They  are  not 
infrequently  taken  for  cases  of  anxiety  neurosis  or  other  neurasthenoid 
hybrids.  Severe  gastric  or  enteric  crises  have  led  to  a  mistaken 
diagnosis  of  tabes.  Undeveloped  forms  may  be  readily  overlooked, 
especially  when  the  more  classical  symptoms  just  noted  are  not  present. 

Particular  attention  should  be  focussed  on  the  thyroid  itself.  Its 
rich  and  increased  vascularity  tends  to  give  it  a  peculiar  consistency, 
even  when  not  markedly  enlarged,  which  is  very  characteristic.  Kocher 
has  compared  it  to  the  general  fulness  of  the  breast  of  a  pregnant  or 
nursing  woman. 

In  a  very  large  number  of  patients,  especially  those  showing  the 
sympathetic  irritation  (vagotonic)  signs  already  discussed,  hyper- 
trophy of  other  lymphatics  is  to  be  observed.  These  are  chiefly 
to  be  sought  in  the  thymus,  tonsils,  tongue  and  rectal  lymphatics. 
There  is  a  tendency  to  elongated  extremities,  scanty  beards  in  men 
and  badly  developed  genitals  in  women.  Marked  lymphocytosis  is 
also  present.  This  relative  lymphatism  possibly  plays  a  very  im- 
portant compensatory  role  in  the  problem  of  the  disease  and  points 
to  another  subdivision  of  the  malady.     The   patients   with  marked 


EXOPHTHALMIC  GOITRE  151 

psychical  signs  are  apt  to  sliow  both  autonomic  and  sympatlietic 
symptoms.  Certain  patients  show  only  cardiovascular  signs.  These 
are  those  described  as  goitrous  heart  cases.  They  show  tachycardia, 
dilated  heart,  some  respiratory  arrhythmia.  The  eyes  are  often  shiny, 
pupils  dilated,  and  striking  even  if  not  protruded.  Dermographia 
is  frequent  and  dizziness  is  often  complained  of.  Other  closely  related 
forms  suffer  from  dyspnea  and  bronchial  catarrh,  bleeding  from  the 
nose  and  congestion  of  the  upper  air  passages.  Neurotic  goitrous 
heart  from  pressure  is  another  special  type  often  overlooked.  There 
is  also  one-sided,  unilateral  mydriasis,  at  times  tachycardia,  and 
protrusion  of  the  eye  on  the  pressure  side  comes  and  goes.  Rarer 
cases  are  disguised  under  mild  diabetics,  and  F.  Miiller  has  described 
a  group  of  pseudosclerosis  cases  of  hyperthyroidism. 

The  iodine  toxic  cases  form  another  group.  Running  from  the 
nose,  bronchial  catarrh,  salivation,  stomachic  distress,  nausea,  diarrhea, 
sleeplessness,  headache,  and  skin  eruptions  are  the  more  frequently 
found  symptoms  which  may  develop  with  but  small  doses  of  iodides. 

That  the  blood  of  patients  with  exophthalmic  goitre  contains  an 
increased  adrenalin  content  which  in  some  cases  may  be  experimentally 
demonstrated  in  animals,  is  a  point  of  hypothetical  diagnostic  value. 

Occurrence  and  Course.— The  disease  is  comparatively  rare.  The 
proportion  of  men  to  women  is  1  to  6  on  the  average.  Between  fifteen 
and  thirty  are  the  most  frequent  years  of  incidence. 

The  course  is  usually  chronic  with  ups  and  downs  and  many  varia- 
tions. Emotional  shocks  almost  invariably  bring  on  an  attack  or 
increase  an  existing  one.  Acute  infections  frequently  bring  on  exacer- 
bations, while  pregnancy  frequently  acts  advantageously.  The  length  of 
time  that  the  disease  persists  is  extremely  variable — from  three  months 
to  thirty  years.  The  prognosis  also  varies  with  the  severity  of  the 
hyperthyroidism.  With  healthy  individuals  the  prognosis  is  relatively 
good,  with  distinctly  nervous  (especially  vagotonic)  individuals  it  is 
less  hopeful. 

Lymphocytosis,  with  normal  numbers  of  white  cells,  is  a  better 
prognostic  sign  than  lymphocytosis  with  leukopenia. 

Treatment. — This  may  be  surgical  or  by  internal  remedies.  Surgical 
treatment  is  the  most  radical.  The  statistics  of  various  operators 
has  shown  improvement  in  from  6  to  76  per  cent.,  death  in  from  2  to 
22  per  cent.^  Kocher  has  reported  76  per  cent,  good  results.  These 
figures  are  probably  high,  if  ultimate  results  are  meant.  The  operation 
of  choice  is  the  successive  elimination  of  thyroid  substance,  with  min- 
imal handling  of  the  gland.  This  is  advocated  particularly  by  Kocher, 
whose  lethal  results  have  been  from  3  to  7  per  cent.  Death  frequently 
results  in  narcosis,  with  a  very  characteristic  symptom-complex. 
The  face  gets  red,  the  whole  body  becomes  tremulous  and  breaks 
out  in  perspiration.     Diarrhea  supervenes,  the  temperature  rises  and 

lEppinger,  loc.  cit.,  p.  70. 


152  THE  ENDOCRINOPATHIES 

the  heart  action  becomes  excessively  rapid,  and  death  with  cyanosis 
and  dyspnea  takes  place.  Status  thyreolymphaticus  is  possibly 
responsible  for  these  results. 

The  sympathetic  operations  are  not  to  be  recommended.  They 
help  the  eye  symptoms  possibly,  but  the  disease  is  not  one  of  the 
cervical  sympathetics  alone. 

Internal  therapy  is  still  unsatisfactory.  Rest  in  bed  is  primary 
and  essential.  Any  remedy  increasing  the  thyroid  secretion  is  bad, 
hence  thyroids  and  iodine  are  to  be  avoided.  Thymus  has  been 
tried,  with  best  results  in  the  sympathetic  types.  The  fresh  gland 
is  given  by  mouth.  a;-ray  treatment  as  at  present  developed  is 
justified  in  a  limited  number  of  carefully  chosen  cases.  The  chronic 
infectious,  hyperplastic  goitres  do  best  with  light  therapy. 

Pharmacological  agents  which  act  to  diminish  the  thyroid  secretion 
have  been  used.  Chief  of  these  is  belladonna.  It  is  the  most  reliable 
of  the  internal  remedies  and  can  be  given  in  fairly  large  doses.  Adren- 
alin in  solution  by  rectum  is  of  service  at  times.  Vagotonic  cases 
react  best  to  its  influence,  the  tachycardia  and  diarrhea  being  well 
influenced  by  it. 

The  digitalis  group  of  glucosides  are  not  to  be  recommended,  neither 
is  iron  of  any  service. 

Arsenic  and  bromides  may  be  of  passing  service,  especially  the 
latter  in  aiding  sleep. 

Psychotherapy  is  above  all  of  great  value,  especially  as  applied 
toward  an  education  of  the  patient  toward  his  fears  in  the  sense  of 
Dubois.  Psychoanalysis  is  of  the  most  signal  service  in  a  large  group 
of  cases. 

Serum  treatments  aiming  to  exert  a  lytic  action  upon  the  secretory 
cells  of  the  thyroid  have  been  devised.  The  most  promising  are  those 
of  Rogers  and  Beebe. 

PARATHYROID  SYNDROMES. 

Tetany. — Corvisart  first  termed  this  "tetanic"  in  1852.  Frankl- 
Hochwart,  in  1887,  clarified  this  conception,  and  since  his  striking 
description  the  term  tetany  has  had  universal  recognition. 

It  is  highly  probable  that  tetany  must  be  regarded  as  an  example 
of  hyperfunctioning  of  certain  peripheral  parts  of  the  motor  mechanism 
in  which  altered  muscular  excitability  is  secondary,  while  special 
vegetative  neurological  features,  namely,  parathyroid  metabolism, 
plays  the  primary  role. 

Symptoms. — Tetany  has,  as  its  main  symptoms,  tonic,  intermittent, 
bilateral,  often  painful  cramps,  which,  without,  for  the  most  part, 
any  loss  of  consciousness,  involve  the  muscles  of  the  upper  extremities, 
particularly  the  hand,  which  is  held  in  the  obstetrical  position.  The 
muscles  of  the  lower  extremities  may  also  be  involved,  those  of  the 
larynx,  of  the  face,  and  of  the  jaw,  seldom  those  of  the  chest,  abdomen. 


PLATE    V 


Objects  Removed  from  Stomach   of  Patient  Suffering  from 
Gastric  Tetany.     (Warbasse.) 


TETANY  153 

neck,  diaphragm,  or  tongue.  In  rare  cases  the  eyeball  muscles  are 
implicated,  as  is  also  the  bladder.  In  the  sensory  sphere  paresthesia 
and  pains  are  present,  while  hyperesthesia  occurs  now  and  then. 
Pressure  upon  the  brachial  plexus  may  give  rise  to  an  attack  (Trous- 
seau) ;  hyperexcitabilit}'  to  electrical  currents  is  present  (Erb) ;  mechan- 
ical hyperexcitability  of  the  muscles  and  motor  nerves  is  observed 
(Chvostek),  while  the  sensory  hyperactivity  to  mechanical  and 
electrical  stimuli  is  also  present  (Hoffmann).  The  psyche  is  rarely 
uninvolved,  and  following  operative  removal  there  has  developed 
extreme  anxiety  with  the  sense  of  impending  dissolution. 

In  chronic  and  repeating  forms  secretory  and  trophic  disturbances 
occur,  such  as  increased  perspiration,  reddening  of  the  skin,  swellings 
of  the  joints,  mild  edema,  falling  out  of  the  hair  and  nails,  discoloration 
of  the  skin,  urticaria,  and  herpes.  Dyspnea  may  intervene;  polyuria 
and  glycosuria  are  rare  accompanying  symptoms.  Abortive  and  incom- 
plete forms  have  been  designated  "tetanoid"  by  Frankl-Hochwart. 

For  didactic  purposes,  Frankl-Hochwart  divides  tetany  into  simple 
and  acute  forms  and  chronic  recurring  forms.  A  further  division 
of  forms  occurring  in  children  and  in  adults  is  made.  Tetany  of  the 
adult  he  groups  into  seven  classes:  (1)  Tetany  idiopathica — tetany  of 
otherwise  healthy  individuals — workman's  tetany.  This  is  the  form 
which  seems  to  occur  epidemically  as  an  acute,  or  acutely  recurring 
affection  in  certain  cities,  notably  Vienna,  Heidelberg,  etc.,  principally 
in  the  early  spring  months,  and  among  certain  handworkers — tailors, 
shoemakers,  etc.  (2)  The  tetany  of  gastric  and  intestinal  affections. 
(3)  The  tetanies  of  acute  or  infectious  diseases,  typhoid  fever,  cholera, 
measles,  scarlet  fever,  etc.  (4)  The  tetanies  of  acute  poisoning, 
chloroform,  morphine,  ergot,  phosphorus,  renal,  and  genital  substances. 

(5)  The  tetanies  of  maternity  (pregnancy,  parturition  and  nursing). 

(6)  The  tetanies  of  parathyroid  involvement.  (7)  The  tetanies  of 
other  nervous  diseases,  exophthalmic  goitre,  brain  tumors,  cysticerci, 
syringomyelia,  etc. 

Incidence. — Tetany  in  its  different  manifestations  is  undoubtedly 
rarely  seen,  and  is  even  less  frequently  reported.  In  undeveloped 
phases  the  tetanoid  reaction  is  comparatively  frequent  in  children. 
The  instances  of  gastric  tetany  are  probably  the  most  frequent, 
while  the  pure  epidemic  form  has  not  been  encountered  in  the  United 
States.  In  Griffith's  study  only  77  cases  were  found  recorded,  while 
Howard's  later  collection  brings  the  American  cases  to  154  in  1907. 
The  incidence  in  the  Johns  Hopkins  Hospital  has  been  0.05  per  cent. 
Thus,  so  far  as  clinicians  in  English-speaking  countries  are  concerned, 
tetany  may  be  considered  as  being  infrequently  seen,  but  even  in 
countries  in  which  it  is  thought  to  be  epidemic,  it  is  rarely  observed. 
It  is  undoubtedly  often  overlooked,  and  at  times  confused  with  hysteria. 

Etiology. — It  is  unnecessary  to  go  through  all  the  steps  which  have 
led  to  the  knowledge  that  the  parathyroids  play  an  important  role 
in  the  functions  of  the  body,  and  that  the  peculiar  muscular  hyper- 


154  THE  ENDOCRINOPATHIES 

excitability  seen  more  particularly'^  in  tetany  is  in  some  measure  related 
to  these  glands,  especially  as  a  reaction  to  their  defect  or  deficiency. 
It  is  possible  that  other  anomalies  of  neuromuscular  activities  are 
correlated  with  these  glands.  Lundborg  has  advocated  wide  hypo- 
thetical possibilities;  even  the  peculiar  motor  manifestations  of  a 
group  of  mental  disorders — katatonias  and  their  allies — may  have 
some  light  thrown  on  them  through  this  avenue. 

More  recent  workers  have  not  rested  on  the  apparently  certain 
foundation  that  tetany  is  essentially  a  manifestation  of  perverted 
parathyroid  activity,  but  have  sought  to  bring  the  anomalous  forms 
into  conformity  with  this  conception,  and  further,  to  obtain  a  more 
fundamental  insight  into  the  essential  features  of  the  disturbed  neuro- 
muscular reaction.  Whether  the  work  of  MacCallum  and  Voegtlin^ 
has  definitely  solved  this  problem  is  to  be  determined,  but  it  would 
appear  that  the  essential  factor  has  been  found  in  the  relation  of  the 
parathyroid  to  the  calcium  metabolism  of  the  body,  the  hyperexcit- 
ability  of  the  neuromuscular  apparatus  is  primarily  due  to  a  lack  of 
calcium  in  the  blood,  and  this  is  thought  to  be  due  to  a  relative  or 
absolute  insufficiency  of  the  parathyroid  glands.  Just  how  the  calcium 
exchange  of  the  body  is  controlled  by  the  parathyroids,  and  what  role 
in  such  control  is  played  by  other  glands,  is  not  known. 

The  second  series  of  factors  still  remains  to  be  solved.  Why  is  it, 
if  the  facts  just  quoted  bear  an  essential  relation  to  tetany,  that  the 
disease  reaction  seems  to  be  so  strictly  localized?  Is  this  simply 
bad  observation  on  the  part  of  the  clinicians,  or  are  there  local  con- 
ditions of  diet,  water  supply,  etc.,  that  bring  about  changes  in  the 
intake  of  calcium?  Such  conditions  are  known  to  prevail  for  certain 
infants  fed  on  cow's  milk,  and  have  been  extensively  studied.^ 

Why  do  certain  workers — ^tailors,  shoemakers,  etc. — suffer  and  not 
others?  Is  Frankl-Hochwart's  suggestion  regarding  the  peculiar 
position  adopted  by  these  workers  of  value  in  relation  to  the  blood 
supply  of  the  organ?  What  have  gastric  dilatation,  gastro-intestinal 
disorders,  etc.,  to  do  with  the  calcium  intake?  Are  there  perversions 
of  gastric  chemism  that  favor  gastric  exclusion?  This  must  be 
determined  in  order  to  explain  these  cases,  and  why  should  certain 
gravid  women  develop  a  parathyroid  insufficiency? 

Pathology. — Concerning  the  histological  changes,  the  present 
view  excludes  a  specific  pathology.  The  insufficiency  of  the  para- 
thyroids, be  it  relative  or  complete,  may  be  brought  about  by  a  great 
variety  of  lesions.  Such  have  been  described  by  various  observers, 
and  each,  in  turn,  has  been  considered  of  specific  significance.  These 
in  reality  offer  evidence  in  favor  of  the  parathyroid  insufficiency 
hypothesis,  but  go  no  further.  In  the  minor  grades  of  tetany  in  chil- 
dren, particularly  in  so-called  spasmophiles  which  Frankl-Hochwart 
regards  as  tetany,  the  findings  of  Yanase  of  Escherisch's  clinic  are 

1  American  Journal  of  Insanity,  1909. 

^  See  Escherisch's  Monograph,  Tetanie  der  Kinder,  1909,  for  most  recent  summaries. 


TETANY  155 

illuminating.  Here  hemorrhages  in  the  parathyroid  seemed  fairl\^ 
constant  findings,  and  offer  an  explanation  of  the  galvanic  hyper- 
excitability.  At  the  other  extreme  one  finds  the  absolute  insufficiency 
tetanies  in  experimental  parathyreopriva  and  in  the  rarer  cases,  such 
as  Pool's  in  man.  Here  the  parathyroid  insufficiency  is  established, 
and  the  calcium  treatment  restorative.  In  acute  epidemic  forms 
thyroid  (and  probably  parathyroid)  involvements  are  known.  Tumors, 
tuberculosis  and  a  host  of  other  changes  in  the  thyroids  have  been 
described.  It  will  probably  be  found  that  in  most  of  these  the  para- 
thj^roids  are  likewise  implicated.  Thus,  in  exophthalmic  goitre  a 
combination  of  thyroid  and  parathyroid  symptoms  is  often  present. 
In  many  tetanies  pure  thyroid  symptoms  appear. 

Symptoms. — Considerable  variation  is  to  be  found,  but  in  general 
four  types  of  symptoms  are  observable  in  the  fully  developed  attack. 
These  are  the  muscular  spasms,  which  may  go  on  to  an  exhaustion 
paralysis,  or  paresis;  the  Trousseau  phenomenon;  increased  electrical 
excitability,  or  the  Erb  symptom;  and  mechanical  hyperexcitability 
of  the  muscles — Chvostek's  sign.  In  some  patients  one  or  more  of 
these  may  be  missing.  Abortive  forms,  so-called,  may  present  even 
fewer  signs.  On  the  other  hand,  a  richer  combination  of  symptoms, 
apparently  closely  related  to  the  general  disorder,  may  be  encountered. 
Sensory  disturbances,  anomalies  of  circulation  with  edema,  of  respira- 
tion with  cyanosis,  and  of  temperature  are  sometimes  found.  True 
psychoses,  perhaps  indistinguishable  from  the  hysterical  confusions, 
are  found.  Trophic  disorders  of  the  skin,  hair,  and  nails  occur.  In 
some  rare  instances,  widely  confused  phenomena  resembling  epileptic 
seizures  occur. 

Muscle  Cramps. — The  most  persistent  feature  consists  in  a  character- 
istic form  of  muscular  spasm.  It  occurs  chiefly  in  the  extremities, 
mostly  involving  the  flexors.  The  spasm  is  tonic,  generally  bilateral, 
and  is  usually  induced  by  some  irritation,  mechanical  or  electrical. 
Overexertion,  exhaustion,  changes  in  temperature,  acute  diarrhea, 
or  emotional  excitement  may  precipitate  an  attack.  The  small 
muscles  of  the  hand  are  usually  first  implicated,  perhaps  after  tingling- 
like  prodromata.  There  is  marked  adduction  in  the  interossei  and 
the  thumb.  The  hand  usually  takes  a  very  significant  position- — that 
of  the  "obstetrical  hand."  It  is  also  described  as  a  "penholding" 
position.  In  many  mild  attacks  only  the  thumb  may  be  involved, 
and  in  others  the  hand  alone.  Sometimes  the  hands  are  closed,  making 
a  fist.  Flexion  at  the  wrist  may  follow,  the  arms  then  being  folded 
across  the  chest,  or  they  may  be  held  up  in  the  air  or  down  at  the  side. 
Such  wider  movements  occur  in  the  more  severe  attacks  only.  A 
somewhat  similar  series  of  flexor  cramp-like  movements  may  occur 
alone  or  in  combination  in  the  lower  extremities.  Talipes  equinovarus, 
inversion  of  the  foot,  and  the  plantar  flexion  may  be  present.  The 
legs  may  be  flexed  on  the  thigh,  and  the  thigh  on  the  pelvis  in  the 
most  severe  instances. 


156 


THE  ENDOCRINOPATHIES 


Considerable  emphasis  may  be  laid  on  the  bilateral  character  of 
these  muscular  movements.  It  is  extremely  rare  in  the  true  tetany 
reaction  to  find  one  side  alone  involved. 


Fig.  52. — Tetanic  spasm,  showing  flexion  of  wrist.     (Pool.) 


Fig.  53. — Tetanic  spasm,  showing  plantar  flexion  of  foot  and  toes.      (Pool.) 


These  muscular  cramps  persist  for  a  very  variable  length  of  time. 
Not  only  do  they  vary  in  different  individuals,  but  the  same  patient 
in  different  attacks,  or  at  different  times  in  the  same  attack  may  show 


TETANY 


157 


marked  variation.  In  the  majority  of  cases  reported  the  spasms 
persist  for  from  fifteen  minutes  to  an  hour,  and  two  or  three  hours 
is  not  an  excessive  period.  Hoffmann  has  reported  a  persistent  cramp 
which  lasted  for  ten  days.  In  fatal  cases  the  contractions  pass  over 
into  a  lethal  continuous  spasm. 

Clonic  spasms  are  rare,  but  are  known  as  blepharospasm,  spasms  of 
the  tongue,  etc.  Postconvulsive  paralysis  or  paresis  is  an  uncommon 
outcome.     Excessive  muscular  tire,  however,  is  not  rare. 

Course. — Clinicians  have  recognized  arbitrarily  three  groups  of  cases 
in  adults,  and  most  modern  authors  are  inclined  to  follow  Trousseau 
in  his  classical  description.  In  the  benign  form  the  sensory  phenomena, 
such  as  formication  or  a  simple  sensation  of  heat,  may  precede  the 
spasms.     These  are  confined  for  the  most  part  to  the  hands  or  occasion- 


FiG.  54. — Method  of  producing  tetanic  spasm  of  hand  by  stretching  the  brachial 
plexus  by  forcible  abduction  of  the  arm.     Note  "obstetrical"  hand.     (Pool.) 


ally  to  the  feet.  The  contractures  may  be  fleeting,  persisting  for  from 
five  to  fifteen  minutes,  or  they  may  persist  for  an  hour  or  more.  Often 
the  attack  terminates  by  a  recurrence  of  the  sensory  symptoms.  A 
period  of  repose  lasting  for  a  quarter  of  an  hour  to  two  or  three  hours 
supervenes,  and  the  spasms  recur.  In  some  instances  two  or  three 
attacks  a  day  may  persist  for  several  months.  These  mild  attacks 
may  recur  at  yearly  intervals,  or,  as  in  some  of  Frankl-Hochwart's 
cases,  several  years  may  elapse,  and  then  they  will  recur.  These 
benign  cases  are  usually  unattended  with  much  pain.  Consciousness 
is  not  disturbed,  and  there  are  usually  no  sensory,  trophic,  or  temper- 
ature disturbances.  Again,  more  severe  attacks  are  observed.  These 
occur  more  often  in  young  adults,  the  benign  forms  having  been 
mostly  observed  among  children.     Here  the  contractions  are  more 


158  THE  ENDOCRINOPATHIES 

violent  and  more  painful.  The  preceding  sensory  phenomena  are 
usually  more  pronounced,  and  with  the  increase  in  the  severity  of  the 
condition  other  symptoms  may  be  noted.  Headache,  malaise,  and  a 
rise  in  temperature  of  1°  to  3°  may  be  noted.  The  affected  muscles 
may  show  signs  of  congestion,  and  localized  edema  of  the  hands  and 
feet  may  be  observed.  Other  muscles  than  those  of  the  extremities 
may  be  involved.  The  muscles  of  the  abdomen  may  be  frequently 
contracted  or  the  sternocleidomastoid  and  the  pectorals;  strabismus 
may  be  noted,  either  the  internal,  or  the  external  rectus,  particularly 
the  latter,  being  involved.  Trousseau  first  noted  the  spasms  of 
the  larynx,  the  pharynx,  the  bladder,  and  the  muscles  of  respiration, 
a  severe  dyspnea  supervening  in  the  event  of  the  latter  muscles  being 
affected. 

These  severe  attacks  are  rarer  than  the  benign  ones.  Frankl- 
Hochwart  has  shown  that  there  is  a  distinct  tendency  for  the  well 
marked  lighter  cases  in  many  instances  to  become  graver,  and  the 
good  prognosis  which  most  writers  have  given  is  now  seriously  doubted 
by  this  observer. 

In  the  grave  form  there  is  no  addition  of  symptoms.  The  attacks 
occur  with  greater  and  greater  frequency  and  become  more  and  more 
intense,  and  the  patients  die  as  a  direct  result. 

Diagnosis. — The  diagnosis  of  a  classical  case  offers  few  difficulties. 
In  English  speaking  countries  it  is  apt  to  be  overlooked,  although  the 
more  frequent  reports  of  recent  years  point  to  the  fact  that  it  is  being 
recognized  more  often,  especially  in  its  milder  forms.  The  presence 
of  cramps  in  the  upper  extremities,  alone  or  in  conjunction  with  the 
upper  limbs,  with  the  classical  obstetrical  hand  and  the  additional 
evidence  supplied  by  the  Chvostek,  Trousseau,  and  Erb  signs,  is 
usually  sufficient  to  determine  a  diagnosis.  Frankl-Hochwart  would 
separate  the  different  forms,  saying  that  gastric  tetany  often  offers 
particular  difficulties,  especially  as  a  group  of  individuals  exists  in 
whom  with  gastro-intestinal  disturbances  there  is  a  tendency  to  cramp- 
like contractures  with  the  typical  hand  position.  He  groups  them  with 
the  hysterias,  rather  than  with  the  tetanies,  however. 

Tetany  strumipriva,  or,  better,  parathyreopriva,  as  suggested  by 
Erdheim,^  offers  the  most  classical  manifestations  of  the  disorder, 
throwing,  as  well,  considerable  light  upon  some  of  the  possible  under- 
lying and  fundamental  features  of  this  peculiar  reaction  type.  Insufiic- 
iency  of  the  parathyroids  results  in  convulsive  phenomena  of  the  tetany 
type. 

Prognosis. — The  point  of  view  here  maintained  precludes  the  possi- 
bility of  the  statement  of  a  general  prognosis.  Very  little  is  known 
definitely  of  the  prognosis  in  infants  and  children.  Most  authors 
agree  in  giving  a  fairly  good  prognosis,  although  Frankl-Hochwart 
says  that  healthy  children  rarely  acquire  convulsions,  and  that  the 

1  Mitt.  a.  d.  Grenzgebiet.  d.  Med.  u.  Chir.,  1906,  vol.  xvi. 


TETANY  159 

prognosis  is  not  good.  In  many  of  these  children  only  one  tetany- 
like  spasm  has  been  noted .  In  others  the  spasms  may  persist  for  weeks 
and  even  months.  In  simple  cases  the  prognosis  is  much  better 
than  in  those  complicated  especially  with  gastric  or  intestinal  affections. 
Bronchitis,  pneumonia,  and  occasionally  an  ascaris  infection  also 
determine  a  less  favorable  prognosis.  Dangerous  signs  appear  with 
glossal  cramps,  which  may  cause  death.  Recurrences  are  frequent 
in  those  who  recover. 

Tetany  coming  on  during  pregnancy  and  childbirth  usually  has 
a  good  prognosis.  The  hyperexcitability  of  the  nervous  system  may 
persist  for  weeks  after  delivery.  In  succeeding  pregnancies  the  re- 
currence of  the  phenomenon  may  be  looked  for.  Frankl-Hochwart 
notes  that  in  succeeding  pregnancies  the  attacks  are  apt  to  be  milder. 
It  should  be  borne  in  mind,  however,  that  sudden  death  may  occur 
in  these  cases.     The  classical  cases  of  Trousseau  and  Szukitz  are  cited. 

In  the  cases  apparently  due  to  disturbances  of  the  stomach  surgical 
interference  has  brought  about  distinct  amelioration.  Sudden  death 
may  occur,  and  apart  from  surgical  intervention  the  prognosis  is 
admittedly  bad  (70  to  80  per  cent.).  The  cases  are  comparatively 
rare,  however.  In  severe  cases  associated  with  marked  gastric  dila- 
tation, operation,  if  only  exploratory,  is  advisable.  The  mortality 
after  operation  in  some  dozen  or  more  cases  now  reported  is  as  low  as 
30  per  cent.  The  subsequent  history  of  these  patients  remains  to  be 
reported. 

Tetany  following  infectious  diseases  and  acute  or  chronic  poisoning 
seems  to  present  a  favorable  prognosis,,  perhaps  the  best  of  the 
various  forms. 

The  prognosis  of  tetany  thyreopriva  depends  upon  the  amount 
of  thyroid  gland  removed  and  whether  the  parathyroids  are  included. 
Total  extirpation  of  the  entire  thyroid  and  parathyroid  tissue  is 
recognized  to  have  a  fatal  outcome.  Tetany  appears  after  total 
removal  of  the  parathyroids,  not  only  in  man,  but  in  lower  animals. 
What  the  outcome  in  man  may  be  of  complete  parathyroidectomy 
with  the  retention  of  thyroid  tissue  is  not  yet  satisfactorily  determined. 

The  most  important  class  numerically  is  the  so-called  epidemic 
tetanies.  Frankl-Hochwart,  in  his  earlier  papers,  gave  a  fairly 
good  prognosis  in  this  form,  but  in  later  contributions  he  presents  the 
further  histories  of  these  patients,  and  finds  that  the  prognosis  is  far 
from  being  good. 

Treatment. — From  the  standpoint  here  outlined  it  may  be  readily 
deduced  that  a  general  treatment  is  not  a  rational  mode  of  approach. 
The  parathyroJdectomized  individual  would  not  be  benefited  by  a 
gastric  operation.  The  essential  feature  in  the  general  treatment  is 
the  search  for  the  irritant,  and  this  cannot  always  be  successful. 

In  approaching  any  specific  instance  the  first  question  is  as  to  the 
validity  of  the  phenomenon.  This  is  placed  first,  because  it  should  be 
the  simplest  factor  to  exclude.     If  no  reason,  remote  or  apparent. 


160  THE  ENDOCRINOPATHIES 

can  be  found  which  would  account  for  simulation,  aggravation,  or 
suggestion,  it  may  be  assumed  that  the  excitation  of  the  nervous 
system  has  other  than  a  purely  psychogenic  origin.  In  rare  instances, 
simulation  has  been  a  factor;  thus,  patients  have  simulated  tetanoid 
contractures  in  order  to  obtain  the  pleasure  of  chloroform  seminarcosis, 
and  continue  a  habit  once  acquired.  Hospital  parasitism  counts 
among  its  devotees  a  number  who  find  the  tetanoid  spasm  a  source  of 
interest  and  advantage;  but  these  cases  are  not  common. 

With  a  positive  diagnosis  established,  the  organ  involved  should 
come  into  review.  Inasmuch  as  parathyroid  insufficiency  is  the  most 
general  cause,  it  is  rational  to  treat  those  cases,  many  in  children,  the 
whole  group  of  so-called  idiopathic  tetanies,  many  tetanies  of  pregnancy 
and  of  thyroid  disease,  by  thyroid  and  parathyroid  preparations. 
Parathyroid  preparations  seem  to  fulfill  most  of  the  conditions,  yet 
occasionally  the  combined  thyroid  and  parathyroid  involvement 
renders  the  giving  of  the  combined  products  of  more  service. 

The  use  of  foodstufl^s  rich  in  calcium  and  of  calcium  salts  follows  as 
a  natural  corollary  from  the  studies  enumerated.  For  the  most  part, 
it  would  seem  that  such  medication  might  entirely  replace  the  use  of 
the  glandular  substances  themselves.  In  experimental  tetanies  the 
successful  effect  of  the  calcium  salts  has  been  very  striking,  and  in 
tetanies  in  children  calcium  therapy  has  given  almost  uniformly 
good  results.  Such  therapy  apparently  renders  the  older  means 
unnecessary,  such  as  curare,  opium,  hyoscyamus,  the  bromides,  chloral, 
belladonna,  chloroform,  galvanism,  sweat  baths,  etc.  Up  to  the 
present  time  therapeutic  experience  is  not  sufficient  to  definitely  prove 
the  durability  of  calcium  medication  in  the  cases  in  which  it  seems 
needed. 

The  surgical  expedient  of  transplanting  parathyroid  tissue  has  proven 
successful  in  animal  work;^  its  successful  application  in  persistent 
chronic  tetanies  in  man  is  clearly  foreshadowed  by  the  experimental 
work  on  dogs.  The  technical  difficulties  do  not  seem  insuperable  in 
view  of  the  ready  transplantation  of  these  structures  in  different  parts 
of  the  body. 

Inasmuch  as  comparatively  definite  light  has  been  thrown  on 
the  whole  subject  of  the  tetany  reaction,  by  the  researches  of 
MacCallum  and  Voigtlein  in  particular,  the  stomach  tetanies,  hereto- 
fore a  particularly  obstinate  group,  offer  opportunity  for  a  combined 
treatment,  radical  as  well  as  conservative.  Useless  gastric  operations 
need  not  be  performed,  yet  at  the  same  time  the  lines  are  laid  down 
that  point  to  the  time  when  such  operations  may  be  of  life  saving 
service.  When  obvious  gastro-intestinal  conditions  point  not  only  to 
such  irritants  in  the  cycle,  it  were  folly  to  persist  in  a  line  of  medication 
which,  notwithstanding  its  theoretical  possibilities,  does  not  give 
results.    Gastrostomy,  gastrectomy,  gastroduodenostomy,  etc.,  accord- 

'Leischner,  Arch.  f.  klin.  Chir.,  1907,  Ixxxiv.  1,  20S. 


DISEASES  OF   THE  HYPOPHYSIS— PITUITARY  161 

ing  to  the  gastric  lesion,  may  be  demanded,  since  the  gastric  intestinal 
mucosa  seems  to  be  an  important  factor  in  the  possibihty  of  gastric 
intake. 

The  best  methods  of  administration  of  calcium  salts  are  matters 
of  individual  experience.  Foodstuffs  rich  in  calcium,  milk,  eggs, 
whey,  etc.,  are  clearly  useful  forms.  Injections  of  calcium  chloride, 
which  have  been  useful  in  animal  work,  may  be  adopted  in  man. 
Calcium  hypophosphites  or  other  salts  to  be  taken  by  mouth  are 
available,  and  intestinal  enemata  are  indicated  if  other  avenues  of 
medication  are  contra-indicated.  The  exact  dosage  remains  to  be 
determined. 


DISEASES  OF  THE  HYPOPHYSIS— PITUITARY. 

Clinically  three  main  trends  may  be  recognized  which  are  due  to 
increased,  diminished  or  irregular  functioning  of  different  parts  of  the 
pituitary  structures.  The  analyses  of  these  syndromes  has  only 
just  begun,  but  in  view  of  Tilney's  fundamental  studies  on  the  hypo- 
physeal structures,  a  definite  classification  will  probably  develop: 
(1)  Hyperpituitarism  is  associated  with  gigantism  and  with  acro- 
megaly; (2)  hypopituitarism  with  various  grades  of  infantilism,  physical 
and  mental,  with  adiposity  and  genital  dystrophies;  (3)  dyspituitarism 
shows  many  mixed  syndromes. 

Hyperpituitarism,:  Acromegaly  and  Gigantism. — These  conditions 
are  apparently  closely  related  and  intermingled,  showing  overgrowth 
in  the  skeleton  and  particularly  in  the  long  bones  in  gigantism;  changes 
in  the  toes,  fingers  and  bones  of  the  face,  more  prominent  in  the 
acromegalic  tendency.  In  general  gigantism  occurs  when  the  disorder 
begins  prior  to  epiphj^seal  union,  acromegaly  when  the  changes  occur 
after  the  union  of  the  epiphyses.  Prodromata  such  as  fatigue,  mus- 
cular pains,  apathy  and  sleepiness  are  frequent. 

The  growth  in  acromegaly  is  very  gradual.  It  includes  changes 
in  the  skin  and  hair  as  well  as  in  the  bones.  These  are  all  hyper- 
trophied,  causing  marked  variation  from  the  usual.  The  eyebrows 
are  heavy  and  overhanging,  with  coarse  hair;  the  lips  are  thickened 
and  protrude,  with  marked  projection  of  the  lower  jaw.  The  mucous 
membranes  share  in  the  hypertrophy.  The  hands  and  feet  are  notably 
widened,  the  fingers  and  toes  stumpy  and  thick.  The  skin  and  hair 
throughout,  inclusive  of  the  genitals,  show  the  same  hypertrophies, 
as  do  practically  all  of  the  bones  of  the  skeleton.  Amenorrhea  is 
frequent  in  women  and  loss  of  potency  in  men.  Glycosuria  is  frequent. 
Carbohydrate  tolerance  may  be  high,  however,  and  an  increased 
fondness  for  sweets  is  frequent. 

Symptoms. — In   addition  to  the   essential   metabolic  disturbance, 
symptoms  due  to  the  nature  of  the  producing  lesions — tumor,  hyper- 
plasia, i.  e.,  neighborhood  symptoms,  are  frequently  found,  but  these 
are  not  invariable. 
11 


162  ,  THE  ENDOCRINOPATHIES 

Severe  bitemporal  headaches  are  frequent.  This  is  an  intracranial 
pressure  sign.  The  sella  turcica  is  usually  enlarged  from  tumor 
formation,  and  this  is  readily  seen  by  a:-ray. 

Pressure  upon  the  optic  nerves  at  the  chiasm  is  usual,  leading  to 
various  types  of  hemianopsia.  Distorted  fields  are  the  rule.  Pupillary 
phenomena  are  frequent,  and  visual  defects  are  often  serious  and 
progressive. 

Mental  symptoms  ranging  from  sluggishness  to  complete  deteriora- 
tion occur,  but  are  not  invariable. 

Other  of  the  endocrinous  glands  may  show  changes. 


Fig.  55. — Characteristic  hand  of  acromegaly.  Note  heaping  of  tissues  about  nails, 
"type  en  large"  of  Marie.  Compare  with  Fig.  56.  (From  Cushing's  "Pituitary 
Body.") 

Prognosis. — This  is  always  grave.  The  disorder  is  progressive,  usu- 
ally very  gradual,  five  to  twenty  years,  but  the  advance  in  symptoms 
may  be  arrested. 

Hypopituitarism. — Deficiency  of  the  hypothetical  pituitary  substance 
gives  rise  to  a  group  of  syndromes  the  most  classical  of  which  is 
Frohlich's  dystrophia  adiposogenifalis.  This  is  characterized  by  a 
progressive  accumulation  of  fat  throughout  the  body,  chiefly  showing 
about  the  buttocks  and  breasts,  and  by  a  lack  of  development  of  the 
primary  and  secondary  sexual  characters. 

Thus  there  is  a  stunting  of  the  growth  physical  and  mental,  a 
hypoplasia  genitalis,  and  a  failure  to  develop  the  voice,  the  mammae 
and  the  scrotum  or  testicle.     As  a  rule  there  is   a  fairly  persistent 


DISEASES  OF   THE  HYPOPHYSIS—PITUITARY 


163 


though  shght  subnormal  temperature,  a  marked  degree  of  sugar 
tolerance,  slowed  pulse  and  a  tendency  to  sluggishness  or  even  sleepi- 
ness. Taper  fingers  are  a  contrasting  picture  to  the  pudgy  ones  of 
acromegaly. 

Symptoms. — Neighborhood  symptoms  may  also  be  observed  as  with 
the  acromegalic  s3^mptoms.  The  optic  nerve  changes,  bitemporal 
hemianopsia,  are  among  the  most  important.  Other  symptoms  of  a 
general  nature,  as  headache,  nausea,  vomiting,  changes  in  the  sella 
turcica,  etc.,  often  occur,  especially  from  tumors  which  destroy  the 
hypophysis. 


Fig.  56. — Typical  tapering  hand  of  adolescent  hypopituitarism.  Compare  with  Fig.  55. 
(From  Cushing's  "Pituitary  Body.") 


It  is  by  no  means  clear  as  yet  whether  the  theory  of  a  hormone  is 
an  adequate  explanation.  The  theory  of  action  through  the  vegetative 
nervous  system  is  probably  more  satisfactory. 

Dyspituitarism. — Under  this  head,  the  majority  of  the  anomalies 
due  to  disturbed  pituitary  structures  may  be  gathered.  These  are 
incomplete  forms  of  acromegaly  and  gigantism,  cases  of  adiposity, 
alone  or  with  genital  atrophies,  or  genital  anomalies,  showing  hy- 
])erfunction  or  hypofunction.  Various  epilepsies,  probably  condi- 
tioned by  hydrocephalic  changes  are  examples  of  dyspituitarism. 
\'ariations  in  mental  capacity  are  frequent,  as  well  as  a  variety  of 
anomalies  such  as  increased  sugar  tolerance  or  glycosuria;  slightly 
sub-  or  supra-normal  temperatures,  polyuria,  wakefulness,  irritability 
and  a  group  of  character  anomalies  as  well.     The  skin  is  usually  smooth 


164  THE  ENDOCRINOPATHIES 

and  soft  and  free  from  moisture,  and  the  hair  is  apt  to  be  thin,  fine  and 
scanty. 

Treatment. — Acromegalic  patients,  or  those  showing  pituitary 
syndromes  due  to  evident  tumor  of  the  hypophysis  region,  need  surgical 
intervention,  whether  the  signs  of  hypo-  or  hyperpituitarism  be  present. 
Hypopituitary  and  dyspituitary  cases  without  neighboring  symptoms 


Fig.  57. — Case  of  post-traumatic  hypopituitarism  in  a  child,  with  extreme  adiposity, 
high  sugar  tolerance,  and  epilepsy.  Marked  improvement  with  whole  gland  feeding 
(pituitary).     (From  Cushing's  "Pituitary  Body.") 

of  tumor  may  be  fed  pituitary  extract,  sometimes  to  advantage. 
Certain  stationary  acromegalic  cases  are  benefited  by  this  treatment 
and  others  apparently  are  rendered  stationary. 

Radiotherapy  is  in  general  inefficient. 

The  chief  surgical  modes  of  relief  are:  (1)  sellar  decompression,  for 
hypophj^seal  headaches,  or  to  permit  a  tumor  mass  to  expand  outside 


Polyglandular  syndromes 


mi 


of  the  cranial  cavity;  (2)  partial  removal  of  a  hyperplastic  and  over- 
acting gland;  (3)  partial  removal  for  sake  of  saving  eyesight;  (4) 
subtemporal  decompression  to  relieve  general  brain  pressure  symptoms ; 
(5)  combined  operations;^  (G)  operations  for  glandular  transplantation. 

POLYGLANDULAR  SYNDROMES. 

Under  this  broad  grouping,  a  large  number  of  syndromes,  due  to 
various  glandular  insufficiencies  or  hyperactivities,  may  be  con- 
veniently placed.     French  clinicians  have  studied  them  extensively 


Fig.  58. — Adipose   genital   dystrophy.     Tumor  of  pineal.     (Bailey   and   Jelliffe.) 


as  has  already  been  indicated.  Some  of  the  more  striking  of  these 
syndromes  are  here  briefly  mentioned.  Their  more  extended  considera- 
tion may  be  found  in  the  works  already  cited,  notably  those  of  Falta, 
Lewandowsky,  Gushing  and  Biedl.  Clinically  speaking  they  make  a 
large  and  variable  medley. 

Pineal  Syndrome. — Here  teratomata  are  chiefly  represented.  The 
patients,  usually  young,  show  the  signs  of:  (1)  general  pressure, 
especially  with  quadrigeminal  signs;  (2)  metabolic  anomalies — ^adi- 
posity,  sexual  precocity  and  abnormal  hair  development.^ 

1  See  Gushing,  Pituitary  Body,  Lippineott,  1912. 

^  Bailey  and  Jelliffe,  Tumors  of  the  Pineal  Body,  Arch,  of  Int.  Med.,  December,  1912. 


166  THE  ENDOCRINOPATHIES 

Adiposis  Dolorosa.- — Dercum  first  named  one  of  these  syndromes. 
The  patients  slowly  develop  fatty  deposits,  often  enormous  in  amount. 
This  adiposity  may  show  as  nodular  deposits  (symmetrical  lipomatosis) , 
varying  in  size  from  a  bean  to  an  apple.  They  may  be  circum- 
scribed or  the  adipose  deposits  may  be  generally  diffused  throughout 
the  entire  body.  The  hips,  shoulders,  upper  arm  and  abdomen  are 
predilection  sites.  The  skin  is  tense.  The  fatty  deposits  are  painful 
to  pressure,  especially  at  nodular  points,  and  even  at  times  before 
there  is  much  fatty  infiltration.  Hyperesthesise  and  paresthesise  in 
the  form  of  tingling,  burning,  numbness,  etc.,  are  frequent.  Spon- 
taneous pains  occur  with  some.  They  are  sharp  and  intermittent, 
localized  in  the  skin  or  more  deeply,  and  often  increase  with  motion. 
Asthenia  is  a  marked  sign  and  psychical  alterations  are  the  rule.  These 
latter  are  mostly  in  the  nature  of  depressions  with,  at  times,  suicidal 
ideas,  irritability,  capriciousness  and  other  signs  closely  resembling 


Fig.  59. — Adiposis  dolorosa.    (Dercum.) 

manic-depressive  states  (q.  v.)  with  flight  of  ideas  and  confusions. 
Other  patients  show  marked  deteriorations. 

Various  anomalous  conditions  have  been  observed  with  different 
patients,  such  as  vasomotor  signs  with  edemas,  ecchymoses,  hemor- 
rhages, and  pigmentation.  Hyperidrosis,  anidrosis,  and  trophic  changes 
also  are  recorded.  Various  complications  with  other  members  of  this 
vast  collection  of  chemical  metabolism  anomalies  are  too  numerous  to 
mention. 

The  disorders  usually  progress  slowly  and  var}^  greatly  in  intensity 
from  year  to  year.  The  patients  recover  spontaneously  or  finally  die 
of  intercurrent  disease. 

The  organs  involved  have  been  the  hypophysis  and  the  thyroid 
chiefly,  but  the  exact  relationships  are  still  uncertain.  Hypophyseal 
tumors  are  not  infrequently  found,  ovarian  disease  is  present  in  some, 
vascular  neurotrophic  disorders  (blood  gland  disease)  are  present  in 
still  others. 


POLYGLANDULAR  SYNDROMES  167 

Therapy. — Some  patients  have  been  helped  by  thyroid  feeding, 
others  not.  Surgery  is  needed  in  tlie  tumor  cases.  Other  symptoms 
are  met  on  their  own  indication. 

Genital  Syndromes:  Agenitalism,  Hypergenitalism  and  Hypo- 
genitalism.— Testicular  and  ovarian  syndromes  are  frequent  and  give 
rise  to  a  motley  group.  Aplasias  of  the  genital  glands,  hermaph- 
roditism, eunuchism,  eunuchoidism  (artificial  and  natural,  early  and 
late),  hypergenitalism,  chlorosis,  menopause  irregularities,  osteomal- 
acia, infantilism,  achondroplasia  are  the  more  frequently  utilized 
symbols  now  current. 

Inter-renal  syndromes  may  be  considered  here,  since  the  cells  of 
the  inter-renal  (suprarenal)  and  the  sperm  cells  (gonads)  have  a 
common  origin.  The  chief  syndrome  of  interest  is  that  called  Addison's 
disease.  Here  the  chief  signs  are  great  muscular  asthenia,  gastro- 
intestinal disturbances,  and  pigmentation — bronzing  of  the  skin.  The 
loss  of  adrenalin  is  responsible  and  this  is  under  nervous  (vegetative) 
control. 

Status  Thymolymphaticus. — The  thymus  has  very  close  relationships 
to  the  gonads,  and  it  is  thought  that  the  thymus  and  testes  are  recip- 
rocally acting  organs.  This  does  not  seem  to  be  true  for  the  thymus 
and  the  ovaries. 

Certain  individuals  have  excess  of  thymus  lymph  tissue  throughout 
the  body.  In  recent  years  the  researches  of  Bartel,i  Wiesel  and  A. 
Paltauf  have  shown  that  this  condition  is  very  frequent.  Notwith- 
standing the  fact  that  a  pathological  diagnosis  postmortem  is  easily 
arrived  at  the  clinical  diagnosis  during  life  presents  many  difficulties. 
This  latter  is  largely  due  to  the  circumstance  that  the  diseased  organs 
are  difficult  to  examine  and,  furthermore,  the  signs  of  defective  develop- 
ment which  result  from  the  condition  are  often  very  slight. 

The  recognition  of  status  thymolymphaticus  often  requires  exhaus- 
tive chemical,  physical,  a;-rays,  and  other  forms  of  examination.  Such 
recognition  is  highly  important,  however,  since  these  individuals,  if 
they  may  be  grouped  together,  are  prone  to  react  very  markedly  to 
anesthetics,  bodily  shocks,  infectious  diseases,  and  to  drugs,  particu- 
larly salvarsan,  sera,  and  mercury.  A  large  medley  of  conditions 
accompanying  and  partly  due  to  status  thymolymphaticus,  have  been 
described.    These  may  be  summarized  as  follows: 

1.  General  Signs. — ^As  a  rule  the  length  of  the  body  exceeds  the 
stretch  between  extended  finger  tips.  The  length  of  the  upper  part 
of  the  body  and  of  the  extremities  is  above  the  average.  The  arrange- 
ment of  fatty  tissue  tends  to  make  the  male  resemble  the  female  type, 
and  vice  versa.  The  mental  status  is  inclined  toward  the  infantile. 
Alcohol  resistance  is  very  slight. 

2.  Face. — The  under  jaw  and  the  mastoid  processes  are  under- 
developed and  the  former  results  in  faulty  bite,  anomalies  of  dentition 

1  Status  thymicolymphaticus,  Deuticke,  1912. 


168  THE  ENDOCRINOPATHIES 

by  displacement  and  by  crowding.  The  palatal  arch  is  high;  the 
tonsils  and  tongue  papillse  are  increased  in  size.  The  epiglottis  is 
inclined  to  be  infantile  in  type.  Epicanthus,  eccentric  pupils,  irregu- 
larly pigmented  irides,  adherent  ear  lobules  and  narrow  external 
auditory  meatus  may  be  present. 

3.  Neck. — The  thyroid,  cervical,  and  other  glands  are  enlarged. 

4.  Skeleton. — The  thorax  is  long  and  narrow.  Cervical  floating 
ribs  are  present.  Compensatory  lordosis  of  the  spine  is  lacking. 
The  scapulae  are  wing-shaped.  The  pelvis  develops  heterosexually; 
the  sacrum  is  small,  the  pulse  high.  Hyperdactyly,  flat-foot,  and 
hyperextension  of  the  elbows  may  be  looked  for. 

5.  Hair. — Axillary  and  pubic  hair  are  diminished;  the  extremities 
may  be  hairy. 

6.  The  thymus  is  enlarged,  the  breasts  resemble  those  of  the  oppo- 
site sex;  polymastia  may  be  observed.  The  aorta  is  narrow,  the 
heart  small,  the  blood-pressure  low.  Palpitation  is  frequent  and  there 
is  cardiac  dilatation  with  weakness. 

7.  In  the  abdomen  ptoses  are  frequent.  The  jugulopubic  dis- 
tance is  increased,  the  abdominal  circumference  diminished.  The 
spleen  is  enlarged,  the  kidneys  prolapsed.  There  is  a  tendency  to 
orthostatic  albuminuria  and  to  alimentary  glycosuria. 

8.  The  blood  picture  shows  a  neutropenia,  lymphocytosis,  and 
eosinophilia. 

9.  The  genital  anomalies  are  in  the  nature  of  cryptorchis,  hypo- 
plasia, disturbances  of  menstruation  and  secondary  sexual  characters 
of  the  opposite  sex. 

10.  There  is  a  marked  disposition  to  other  disease  and  usually  a 
tendency  to  an  increase  in  the  severity  of  the  disorder.  Thus,  tuber- 
culosis shows  more  often  in  other  organs  than  the  lungs;  infectious 
diseases  of  childhood  are  severe ;  there  is  a  tendency  to  tetany,  glio- 
mata,  syringoses,  hydrocephalus,  tabes,  paresis,  myasthenia.  Dia- 
betes, excessive  fat  and  gout  occur.  Pernicious  anemia,  leukemia 
and  chlorosis,  exophthalmic  goitre,  Addison's  disease,  osteomalacia, 
nephritis,  eclampsia,  asthma,  infantile  emphysema,  eczema,  heman- 
giomata,  appendicitis  and  tumor  formation  are  among  other  accom- 
panying disorders. 

Observation  of  many  cases  of  status  thymolymphaticus  shows  that 
little  weight  is  to  be  given  to  the  occurrence  of  isolated  symptoms. 
The  diagnosis  consists  in  the  accumulation  of  the  anomalies.  The 
difi^erences  in  body  dimensions  are  of  universal  importance,  whereas 
the  increase  in  the  tongue  follicles  and  the  infantile  character  of  the 
epiglottis  and  its  frequent  omega  shape,  are  more  characteristic. 
Genital  hypoplasias  are  frequently  associated  with  eosinophilia  and 
lymphocytosis  is  to  be  expected. 

The  inter-relationships  of  the  various  endocrinous  glands  may  be 
illustrated  by  the  reproduction  of  the  series  of  charts  or  diagrams  in 
Paton's  admirable  treatise  on  the   Nervous  and  Chemical  Regulators 


POLYGLANDULAR  SYNDROMES  169 

of  Metabolism,  London,  1913.  As  Paton  well  remarks,  these  may 
well  be  a  grotesque  parod}-  of  what  will  ultimately  be  found  to  be  the 
relationship  of  the  activities  of  these  organs.  "They  are  probably  as 
near  the  truth  as  those  quaint  ancient  maps  of  the  Indies  with  their 
'here  be  much  gold'  scrawled  across  them,  which  served  as  the  charts 
of  our  forefathers,  but  if,  like  them,  they  merely  indicate  the  direction 
which  further  investigation  should  take  and  suggest  lines  of  attack, 
they  will  have  served  their  purpose." 

The  direct  and  profound  action  of  the  secretions  of  the  sexual  glands 
(gonads)  upon  the  body  is  seen  in  every  tissue  of  the  body.  How  far 
their  action  is  facilitated  and  how  far  checked  by  other  endocrinous 
organs  is  not  yet  entirely  worked  out.  The  thymus  supplements  the 
action  of  the  testes  secretion.  Its  relations  to  the  ovaries  is  not  so 
certain.  It  exercises  a  checking  action  on  the  male  gonad  activity 
which  in  its  turn  acts  reciprocally  on  the  thymus.    (See  Figs.  60  to  63.) 

The  removal  of  the  thyroid  checks  the  growth  of  the  gonads.  Cas- 
tration acts  less  on  the  thyroid,  although  menstruation,  childbirth, 
and  the  menopause  cause  marked  thyroid  activity.  (See  Thyroid, 
Figs.  60  and  63.) 

The  destruction  of  the  pituitary  leads  to  gonad  atrophy  and  recip- 
rocally castration  causes  hypertrophy  of  the  pituitary.  The  secretion 
of  both  stimulate  the  growth  of  the  long  bones,  the  uncontrolled 
activity  of  the  former  leading  to  gigantism  and  acromegaly.  The 
action  of  the  gonad  secretions  is  to  check  the  pituitary  activity  and 
the  increase  in  size  of  the  eunuch  is  possibly  a  response  to  this 
unchecked  hypophyseal  activity.  The  gonads  are  not  alone  in  hinder- 
ing the  pituitary  action. 

Suprarenal  and  gonad  activity  are  closely  related  and  suprarenal 
loss  is  usually  accompanied  by  genital  aplasias  or  anomalies.  Paton 
has  suggested  the  identity  of  certain  elements  of  these  tissues  and  that 
the  suprarenals  constitute  a  sort  of  bridge  or  intermediary  between 
the  bodily  and  the  sexual  cells. 

The  thyroids  and  pituitary  are  closely  related.  Removal  of  one 
causes  hypertrophy  of  the  other.  (Fig.  60.)  They  thus  mutually 
check  each  other  in  part  and  are  also  cooperative,  the  pituitary  need- 
ing the  thyroid  to  complete  its  activities.  Hyperthyroid  activity 
does  not  lead  to  hyperplasias  of  connective  or  bony  tissues  as  does 
hyperpituitary  action;  the  reciprocal  autonomic  and  sympathetic 
nerve  activity  is  not  exactly  similar;  although  diminished  activity 
of  both  substances  may  lead  to  diminished  bony  growth — atrophy. 
The  vegetative  mechanism  of  this,  however,  has  not  yet  been 
elucidated. 

The  action  of  thymus  on  thyroid  is  far  from  clear,  but  the  tendency 
is  to  show  a  reciprocal  checking  action  especially  on  the  neuromuscular 
apparatus.  The  problem  of  myasthenia  gravis  has  been  thought  to 
lie  behind  this  reaction. 

The  thyroids  and  parathyroids  have  distinctly  different  and  even 


170 


THE  ENDOCRINOPATHIES 


antagonistic  activities :  The  former  seems  to  be  related  more  distinctly 
to  the  iodine,  the  latter  to  the  calcium  metabolism  of  the  body.  Just 
how  they  are  regulated  through  the  vegetative  nervous  system  is 
unknown.    Calcium- is  of  pronounced  value  in  neuromuscular  activity 


Fig.  60. — To  show  the  probable  influence  of  the  various  endocrinous  structures  on 
one  another.     The  following  explanations  apply  to  this  and  to  the  three  succeeding 

figures.     (Paton.)     stimulation;    inhibition.     The   arrow   indicates   the 

direction  of  action.  Hyp.,  hypophysis;  Par.,  parathyroid;  Ch.,  Chromaffin  system; 
Art.,  artery;  Pit.,  pituitary;  Thm.,  Thymus;  Th.,  thyroid;  G.,  glands;  B.,  bone;  In., 
inter-renal;  Pan.,  pancreas;  M.,  muscle.    (Paton.) 

as  the  phenomena  of  tetany  show.  Myasthenic  states  in  general  and 
myasthenia  gravis  in  particular  are  more  directly  related  to  disordered 
thyroid  and  thymus  activities.  Lundborg  has  shown  that  the  para- 
thyroid function  plays  some  part  in  the  reaction. 

The  thyroid  acts  on  the  pancreas  chiefly  through  its  action  on  the 
liver  sympathetic  fibers.    Sugar  mobilization  and  release  are  brought 


Fig.  61. — To  show  the  probable  mode  of  action  of  the  various  internal  secretions  on 
the  mobilization  of  sugar  in  the  liver.      (Paton.) 


about  through  modified  thyroid  and  pancreatic  action,  which  latter 
prevents  the  mobilization  of  sugar  in  the  liver.  Thus  glycosuria  is 
frequent  in  hyperthyroid  states.    (See  Fig.  61.) 

The  complicated  inter-relationships  cannot  be  entered  into  more 


POLYGLANDULAR  SYNDROMES 


171 


fully.  The  chief  available  literature  has  been  indicated.  One  point, 
however,  should  be  emphasized  and  that  is  that  the  activities  of  the 
internal  secretion  organs  are  all  under  vegetative  nervous  system 
control.     The  active  substances,  hormones,  if  one  wishes,  are  not 


Fig.  62. 


-To  show  the  probable  mode  of  action  of  certain  of  the  internal  secretions  upon 
the  spinal  reflex  arc.     (Paton.) 


independent  chemical  activators,  they  are  under  sympathetic  and 
parasympathetic  (autonomic)  control.  The  output  of  iodine,  of 
calcium,  of  adrenalin,  of  hypophysin  and  of  all  of  the  substances 
thus  far  known  or  named  is  controlled  almost  exclusively  by  the 
nervous  system.  The  internal  secretions  act  through  the  nervous 
system.    While  it  may  be  shown  that  within  an  organ  itself  primary 


Fig.  63. — To  show  the  probable  mode  of  action  of  the  internal  secretions  on  the 
growth  of  muscle  and  of  bone  and  other  connective  tissues.  The  possibility  of  this  being 
a  vasomotor  reflex  mechanism  is  indicated  by  lines  marked?     (Paton.) 

chemical  regulators  may  be  effective- — thus  one  must  explain  the  posi- 
tive and  negative  tropisms  within  the  cells  of  an  organ  in  its  initial 
response  to  a  disturbance  of  cellular  adjustment — yet  the  chief  activ- 
ities of  the  internal  secretions  are  brought  about  by  neurochemical 
regulators,  as  Paton  terms  them. 


172 


THE  ENDOCRINOPATHIES 


Just  as  the  complicated  sensori-motor  integrations  are  effective 
in  governing  the  muscular  activities  of  the.  human  body,  so  the 
integration  of  neurochemical  regulators,  taking  place  at  the  physico- 
chemical  level,  is  effective  in  adjusting  the  metabolism  of  the  body 
cells.  Hormones  are  not  the  activators  primarily;  they  are  the  ser- 
vants of  the  vegetative  nervous  system.  All  of  the  endocrinopathies 
are  really  polyglandular  syndromes  and  under  psychical  control. 


Fig.  64. — Scheme  of  innervation  of  the  liver,  spleen,  and  kidney.  nX,  nucleus  of  the 
vagus;  -X",  vagus;  nv,  vasomotor  nucleus  in  medulla;  s,  sympathetic;  re,  rami  commu- 
nicans;  spl,  splanchnic  nerve;  ps,  solar  plexus;  gs,  semilunar  ganglion;  spl,  spleen. 
(Bechterew.) 


Pancreatic  Syndromes. — Falta  holds  that  the  chief  activity  of  the 
pancreas  is  subserved  through  an  assimilatory  hormone,  which  controls 
the  glycogenesis  of  the  liver  and  muscles.  In  mild  grades  of  pancreatic 
insufHciency  disturbances  of  carbohydrate  metabolism  appear  only 
when  great  demands  are  made  upon  the  glycogenic  function  of  the 
liver  through  excessive  alimentary  carbohydrate  intake.  In  graver 
disturbances  in  addition  to  the  modification  of  anabolism  a  high 


POLYGLANDULAR  SYNDROMES 


173 


grade  of  catabolic  destruction  takes  place  with  a  failure  to  form 
higher  and  lower  fatty  acids  (ketonuria). 

Pancreatic  syndromes  occur  as  a  result  of  gross  anatomical  disorder, 
acute  pancreatic  hemorrhage,  and  chronic  pancreatitis,  syphilitic 
pancreatitis,  etc.;  all  of  which  are 
discussed  fully  in  works  on  internal 
medicine.  Those  of  interest  here, 
however,  are  diabetes  mellitus, 
true  diabetes,  and  pancreatic  in- 
fantilism, all  closely  related  to  dis- 
order of  the  chromaffin  tissues  of 
the  pancreas — its  internal  secretory 
part.  Although  the  pancreas  seems 
primarily  a  digestive  gland  it  also 
produces  an  internal  secretion  which 
holds  in  check  the  mobilization  of 
sugar,  thus  acting  in  a  balanced 
relation  with  the  thyroid  and  hj^po- 
physeal  secretions  which  tend  to 
facilitate  the  use  of  sugar  as  an 
energizing  material  by  the  muscles. 
This  mobilization  may  be  con- 
sidered to  be  made  effective  by  the 
terminals  of  the  vegetative  nervous 
system  in  the  liver  cells;  just  how 
it  is  not  known.  Pancreatic  in- 
fantilism, shows  polyglandular  dis- 
turbances, through  arrested  bodily 
growth  and  arrested  sexual  develop- 
ment. Vagotonic  symptoms  such 
as  excessive  diarrhea  and  flatulent 
distention  are  also  present. 

Liver  Syndromes. — Progressive 
Lenticular  Degeneration. — Wilson^ 
has  described  this  syndrome,  to 
which  he  applies  the  term  progres- 
sive lenticular  degeneration. 

The  disease  is  familial,  in  the 
sense  that  frequently  more  than 
one  member  of  a  family  is  affected 
with  it,  but  it  is  not  hereditary; 
it  may  also  occur  sporadically.     It 

occurs  in  young  people,  either  in  an  acute  or  a  chronic  form.  As  far 
as  is  known,  it  is  progressive  and  invariably  fatal,  its  duration  ranging 
from  six  months  or  a  year  to  as  long  as  four  or  five  years. 


Fig.  65.- 


Progressive  lenticular  degener- 
ation.     (Tilney.) 


1  Progressive    Lenticular    Degeneration.      A    Familial    Nervous    Disease,    Associated 
with  Cirrhosis  of  the  Liver.     Brain,  1913. 


174  THE  ENDOCRINOPATHIES 

Syviptoms. — ^The  clinical  symptoms  consist  of  involuntary  move- 
ments, nearly  always  a  bilateral  tremor  of  both  upper  and  lower 
extremities,  the  head  and  trunk  also  being  sometimes  involved.  The 
tremor  is  usually  rhythmical  but  occasionally  irregular,  and  increasing 
with  volitional  movement;  there  is  pronounced  spasticity  of  the 
limbs  and  of  the  face,  the  latter  being  usually  set  in  a  spastic  smile, 
while  in  the  latter  stages  contractures  of  the  limbs  develop;  there  is 
dysphagia  and  dysarthria,  the  latter  eventually  degenerating  into 
complete  anarthria;  there  is  also  spasmodic  laughing  and  emotional- 
ism. As  a  result  of  the  extraordinary  degree  of  stiffness  of  the  mus- 
culature there  is  considerable  difficulty  in  maintaining  equilibrium. 
Little  or  no  true  paresis  or  paralysis  occurs,  however,  inasmuch  as 
most  ordinary  movements  can  be  executed,  although,  it  may  be,  slowly 
and  feebly.  In  spite  of  the  great  degree  of  motor  weakness  and  help- 
lessness, in  a  pure  case  the  abdominal  reflexes  are  present  and  a  double 
flexor  response  is  obtained.  In  other  words,  this  affection,  where  it 
occurs  in  an  uncomplicated  form,  is  an  extra  pyramidal  motor  disease, 
the  importance  of  which  is  apparent  by  reason  of  the  light  it  sheds  on 
such  a  process  as  paralysis  agitans. 

The  chief  pathological  feature  of  the  disease  is  bilateral  symmetrical 
degeneration  of  the  putamen  and  globus  pallidus,  in  particular  the 
former.  This  degeneration  is  a  sequel  to  the  selective  operation  of 
some  morbid  agent  on  the  cells  and  fibers  of  the  putamen  and  lenticular 
nucleus  generally.  The  caudate  nucleus  is  often  somewhat  degenerated, 
but  never  to  the  same  extent,  while  other  large  collections  of  gray 
matter  in  the  immediate  neighborhood  of  the  lenticular  nucleus — 
e.  g.,  the  optic  thalamus,  which  has  partially  the  same  blood  supply — 
is  not  affected  at  all  in  a  pure  case  unless  it  be  indirectly,  and  to  a 
very  slight  extent.  The  morbid  agent  is  possibly  some  form  of  toxin. 
A  constant,  essential,  and  in  all  probability  primary  feature  of  the 
pathology  of  the  disease  is  cirrhosis  of  the  liver,  not  syphilitic  or 
alcoholic;  it  is  multilobular  or  mixed  in  type,  always  pronounced, 
but  presenting  a  varying  pathological  picture  of  necrosis,  fatty 
degeneration,  and  regeneration. 

It  is  probable  that  the  toxin  is  associated  with  the  hepatic  cirrhosis, 
and  may  be  generated  in  connection  therewith.  An  important  analogy 
may  be  drawn  from  the  occurrence  of  "  Kernikterus"  in  certain 
cases  of  familial  icterus  gravis  neonatorum,  where  in  spite  of  the 
universal  bile  staining  of  the  tissues  of  the  body  certain  collections 
only  of  gray  matter  in  the  brain  show  a  marked  avidity  for  the  circulat- 
ing poison,  while  others  do  not.  The  parts  that  are  stained  deeply 
are  in  particular  the  nucleus  lenticularis  and  the  corpus  Luysii  (among 
others),  while  the  optic  thalamus,  for  instance,  is  scarcely  stained  at  all. 

Muscle  Syndromes. — Myasthenia  Gravis. — The  clinical  position  of 
this  disorder  is  very  uncertain.  By  some  it  is  to  be  regarded  as  a 
contrast  picture  to  tetany  and  due  to  vegetative  nervous  disturbance 
conditioned  infpart  by  diminished  parathyroid  activity.      It  has  of 


MYASTHENIA  GRAVIS  175 

late  been  shown  that  the  striped  muscular  system  is  provided  with 
vegetative  fibers  which  undoubtedly  regulate  the  muscular  metab- 
olism.   By  others  it  is  grouped  with  the  muscular  atrophies. 

The  disorder  is  infrequent.  It  was  separated  from  the  progressive 
bulbar  palsies  of  organic  nature  by  Erb  (1878)  and  later  studied  by 
Oppenheim  (1887),  who  termed  it  a  myasthenic  paralysis  without 
anatomical  foundation.  In  1891  Jolly  described  the  characteristic 
electrical  reactions  occurring  in  the  muscles,  termed  the  myasthenic 
reaction.^ 

The  early  symptoms  which  usually  came  on  between  fifteen  to 
thirty  years  of  age,  usually  involve  the  facial  muscles,  particularly 
those  of  the  upper  lid,  causing  ptosis.  Diplopia  from  paresis  of  an 
ocular  muscle  also  may  be  an  initial  symptom.  The  two  often  occur 
together  (Asthenic  ophthalmoplegia).  The  patients  note  the  beginning 
fatigue  of  the  muscles,  which  perhaps  intact  in  the  morning  on  awaken- 
ing, show  fatigue  signs  at  night.  This  muscular  asthenia  then  pro- 
gresses slowly  to  distinct  paresis.  Other  cranial  nerve  innervations 
then  show  a  similar  asthenia.  Difficulties  in  chewing  develop,  -or  of 
swallowing,  or  of  speaking.  The  muscles  of  the  neck  may  also  be 
involved.  Whatever  group  is  involved  the  chief  feature  is  the  great 
fatigue  which  develops  very  rapidly  after  the  use  of  the  muscle. 

Any  muscle  or  muscle  group  of  the  body  may  be  involved.  Dys- 
pnea and  tachycardia  are  among  the  rarities  of  implication  of  the 
respiratory  and  cardiac  muscles.  Sensory  disturbances  are  not  charac- 
teristic.   Pains  may  occur. 

Leukocytosis  is  usually  present.  The  reflexes  are  not  implicated. 
In  some  instances  fatigue  of  the  tendon  reflexes  is  recorded. 

The  chief  feature  is  the  rapidly  developing  fatigue  of  the  muscle. 
This  is  best  demonstrated  by  faradic  stimuli.  These  cause  a  rapid 
loss  in  the  excitability  of  the  muscle  until  it  no  longer  reacts  to  the 
intermittent  faradic  current.  Hoffmann  has  shown  that  this  is  largely 
influenced  by  the  rate  of  the  interruptions.  With  seventy  interrup- 
tions per  second  the  myasthenic  reaction  develops  promptly,  with 
fifteen  it  does  not.  Continuous  faradic  stimulation  produces  a  similar 
myasthenic  fatigue  curve.  This  myasthenic  reaction  seems  to  separate 
the  disorder  from  other  forms  of  muscular  fatigue  such  as  occur  in 
bulbar  palsy,  medullary  syphilis,  multiple  sclerosis,  Addison's  disease, 
exophthalmic  goitre  and  the  fatigue  of  intermittent  claudication. 
There  are  certain  analogies  with  this  last  disorder  which  are  not  yet 
cleared  up. 

Atrophies  develop  in  the  aftected  muscles,  but  there  are  no  definite 
indications  of  the  reaction  of  degeneration.  Certain  transitional 
cases  which  show  relationships  to  distinct  organic  (nuclear)  cases  may 
evidence  electrical  changes  approaching  R.  D.  Fibrillary  twitches 
in  the  affected  muscles  are  not  the  rule,  but  they  have  been  observed. 

1  Oppenheim,  Die  myasthenische  Paralyse,  1901. 


176  THE  ENDOCRINOPATHIES 

Myasthenia  gravis  runs  a  chronic  course  with  at  times  marked 
remissions.  It  has  been  known  to  develop  rapidly  in  three  to  four 
months  with  fatal  issue  in  from  one  to  three  years  arid  on  the  other 
hand  it  has  been  known  to  extend  over  fifteen  to  twenty  years.  The 
outcome  is  usually  fatal,  but  certain  cases  cease  to  progress. 

Little  is  known  of  the  underlying  causes.  Status  thymicolymphati- 
cus is  frequent.  Many  cases  are  associated  with  disorder  of  other 
endocrinous  glands,  chiefly  with  hyperthyroid  states.  Constitutional 
anomalies,  also  often  regarded  as  of  lymphogenic  origin  are  described. 
Nothing  is  known  concerning  the  psychical  states. 

The  pathological  lesions  are  not  constant.  In  the  greater  number 
of  cases  the  muscles  are  swollen,  edematous  and  infiltrated  with  lym- 
phoid cells.  These  changes  have  not  been  interpreted.  It  is  possible 
that  they  are  edemas  due  to  disturbance  of  the  vegetative  nervous 
system  control,  in  which  case  myasthenia  gravis  is  to  be  allied  with 
the  circuniscribed  edemas.  To  know  this  does  not  help  very  much, 
but  it  does  indicate  that  search  must  be  directed  toward  all  causes 
for  vegetative  nervous  system  disturbance,  toxic  and  psychic. 

The  first  important  therapeutic  agent  is  rest;  absolute  and  pro- 
longed. The  second  is  psychotherapy.  Organotherapy  has  been 
tried,  with  as  yet  little  results,  but  it  probably  has  been  entirely  too 
empirically  applied.  Careful  attention  should  be  given  to  a  complete 
survey  of  the  functions  of  all  of  the  endocrinous  glands,  and  if  a  lack 
of  balance  be  found  an  attempt  should  be  made  to  restore  the  balance. 
Artificial  feeding  is  at  times  necessary.  Atropin  has  been  of  service 
occasionally.  Alcoholic  preparations  are  to  be  avoided  as  are  also 
mechanical  forms  of  stimuli,  particularly  severe  massage. 

Thomsen's  Disease.^ — Myotonia  Congenita. — This  is  a  very  rare  dis- 
order. Its  relationships  to  other  nervous  diseases  is  very  obscure. 
It  is  hereditary,  and  is  probably  conditioned  by  a  constitutionally 
inferior  thoracic  autonomic  control  of  the  muscle  metabolism.^  This 
is  often  associated  in  the  affected  families  with  other  signs  of  inferiority; 
neuroses,  psychoses,  tetany,  etc. 

The  chief  anomaly  is  one  affecting  the  muscles.  At  the  beginning 
of  any  voluntary  movement,  the  patient  finds  it  difficult  to  overcome 
a  muscle  hypertonus.  This  makes  the  muscles  stiff  and  unyielding. 
After  repeated  efforts  the  resistance  gradually  disappears  and  in  a 
few  minutes  or  more  the  muscular  activity  becomes  normal.  This 
limbering  up  effect  is  lost  after  a  cessation  of  the  movements.  Any 
group  of  muscles  may  be  affected,  but  the  lower  extremities  are  oftenest 
involved.  This  makes  the  beginning  of  walking  difficult.  In  the 
upper  extremities  a  similar  condition  makes  manual  movements 
difficult.  A  patient  cannot  readily  loosen  his  grasp  of  an  object. 
Talking  and  eating,  etc.,  may  be  similarly  affected.  Changing  the 
tempo  of  a  movement  increases  the  difficulty  and  emotional  stimuli 

1  Thomsen,  Archiv  f.  Psychiatrie,  1892. 

2  S.  de  Boer,  Zeitschrift  f.  Biologie,  1914,  vol.  Ixv. 


T HO M SEN'S  DISEASE  177 

invariably  augment  the  stifPness  and  awkwardness.  Mechanical 
stimuli  cause  welts  to  appear  which  subside  slowly.  Atypical  cases 
are  reported,  in  some  of  which  the  disorder  appears  intermittently. 
(Compare  with  periodic  paralysis.) 

The  pathological  changes  are  slight.  Muscle-cell  hypertrophy, 
analogous  to  that  seen  in  myasthenia  gravis,  is  described. 

The  disorder  begins  early,  is  very  chronic,  is  not  fatal  itself,  nor 
does  it  seem  to  get  well  spontaneously. 

No  therapy  has  been  shown  to  be  effective.  If  the  present  hypothe- 
sis is  of  value  some  results  should  follow  from  polyglandular  therapy. 


12 


PART  II. 

SENSORI-IOTOR  SYSTEMS. 


CHAPTER  V. 
SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES. 

DISEASES  OF  THE  OLFACTORY  TRACT. 

Olfactory. — In  man  the    olfactory   apparatus    has   lost   much   of 
the  importance  it  possesses  in  a  number  of  the  lower  animals.    (For  the 


Fig.  66. — Extent  of  true  olfactory  receptors  on  the  mucous  membrane,      (v.  Brunn.) 

best  recent  discussion  of  the  anatomy  see   Edinger's   Lessons,  8th 
edition,  1911-12.)     Disease  of,  or  pressure  upon,  the  olfactory  nerve 


180 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


£pithcliu?ri- 
I'entnculi.  o/fac 


in  its  peripheral,  thalamic,  or  cortical  portions  results  in  either  diminu- 
tion (hyposmia)  or  loss  (anosmia)  of  smell;  hallucinations,  illusions 
(parosmia,  ismosmia  or  cacosmia),  or  hyperesthesise,  causing  excessive 
sneezing.  Odor  influences  taste  directly  and  by  association  involve 
the  entire  vegetative  nervous  system.  The  difl^erent  parts  of  the 
olfactory  pathways  need  to  be  taken  into  consideration.  The  study 
of  ofactory  hallucinations,  particularly  in  certain  psychoses,  and   in 

certain   tumors  in  or    about   the   frontal 
lobes,  renders  this  of  value. 

The  receptors  for  smell  are  located  in  a 
limited  portion  of  the  Schneiderian  mucous 
membranes.  Changes  in  that  portion  of 
the  membrane,  such  as  occur  in  any  acute 
inflammatory  disease,  coryza,  influenza, 
diphtheria,  etc.,  cause  diminution  or  loss 
of  ability  to  smell.  Albinism  is  usually 
associated  with  loss  of  smell.  Chronic 
inflammatory  processes,  often  accompanied 
by  fetid  odors,  polyps,  frontal  or  maxil- 
lary sinusitis,  lead  poisoning,  usually  bring 
about  unilateral  or  bilateral  loss  of  smell. 
Most  of  the  causes  for  this  mostly  periph- 
eral loss  of  smell  may  be  estimated  by 
direct  inspection.  Certain  directly  acting 
drugs,  cocaine,  etc.,  influence  smell. 

From  the  receptors,  the  pathways  trav- 
erse the  cribriform  plate  and  enter  the 
olfactory  bulb,  forming  synapses  with  the 
mitral  cells.  Lesions  in  and  about  the 
cribriform  plate,  fractures,  meningitis, 
syphilis,  pressure  of  frontal  tumor  may 
determine  a  diminution  or  loss  of  smell; 
possibly  lesions  in  this  portion  of  the 
olfactory  pathway  may  cause  hallucinatory 
odors,  but  this  is  still  debatable.  Certain 
tumors  Ijang  upon  the  orbital  plate  of 
the  sphenoid  and  compressing  the  lobus 
olfactorius  have  seemed  to  give  rise  to 
unilateral  and  bilateral  hallucinations  of 
smell.  (See  Fig.  67.) 
It  is  known  that  certain  sneezing  crises  have  been  determined  by 
tabetic  lesions.  Just  which  parts  of  the  olfactory  tracts  are  involved 
is  not  certain.^ 

Tertiary    neurones    pass    to    the    thalamus    and    to    the    cortex. 
(See  Fig.  68.) 


Fig.  67. — Illustration  of  first 
and  second  neurones  of  the 
olfactorius.  The  first  synapse 
takes  place  in  the  glomeruli 
(mitral  cells).      (Edinger.) 


Klippel  and  L'Hermitte.  Sem.  Med.,  February  17,  1909. 


DISEASES  OF   THE  OLFACTORY   TRACT 


181 


Clinical  correlations  with  disorder  of  this  portion  of  the  olfactory- 
pathway  are  not  certain.  Certain  overaffective  reactions  to  odors, 
disgusts,  nausea,  even  vomitings  from  odors  need  to  be  more  carefully 
sifted  in  this  connection,  especially  in  relation  to  brain  tumor  localiza- 
tions. They  should  not  be  viewed  as  whims  or  fancies  of  hysterical 
patients.  Loss  of  smell  may  result  from  thalamic  lesions,  usually 
homolateral.  The  crossing  of  the  olfactory  pathways  is  incomplete,  and 
takes  place  principally  in  the  anterior  cerebral  commissure  (Fig.  (iS). 


tulbusolfi 


Fig.  68. — Scheme  of  olfactory  paths.  X,  vagus  root  fibers;  ca,  anterior  commissure; 
cm,  mammillary  body;  cp,  fibers  from  nucleus  habenulse  to  posterior  commissure;  fG, 
tract  from  mammillary  body  to  Gudden's  nucleus;  fi,  fasciculus  mammillo-thalamicus; 
fl,  fasciculus  long,  medianus;  fr,  fornix;  ful,  fibers  of  fornix;  gli,  nucleus  habenulse; 
gi,  interpeduncular  ganglion;  gp,  gyrus  pyriformis;  /,  median  lemniscus;  m,  fibers 
from  Gudden's  nucleus  to  substantia  reticularis;  na,  anterior  thalamic  nucleus;  nG, 
Gudden's  nucleus;  nt,  tegmental  nucleus;  nX,  vagus  motor  nucleus;  peE,  ped.  corp. 
mammilaris  from  fillet;  qa,  quadrigemina ;  r,  fibers  from  n-tegmenti  to  cranial  nerve 
nuclei;  re,  radix  lateralis  tractus  olfactorii;  rf,  fibers  of  olfactory  tract  to  trigonum 
olfactorii;  ro,  median  olfactory  tract  root;  s,  fibers  from  interpeduncular  ganglion 
to  tegmental  nuclei;  so,  olfactory  trigone;  th,  optic  thalamus;  tro,  olfactory  tract; 
tt,  tenia  thalami;  x,  fasciculus  retroflexus.     (Bechterew.) 


The  cortical  neurones  end  in  the  cornu  ammonis,  which  is  a  large 
olfactory  association  field.  (See  Edinger,  Ramon  y  Cajal,  Van 
Gehuchten.)  Lesions  here  result  in  peculiar  olfactory  auras,  as  seen 
in  certain  hippocampal  epilepsies  (uncinate  fits  of  Hughlings  Jackson). 
Such  fits  occur  from  temporosphenoidal  tumors  also.  Olfactory  agno- 
sias also  result  from  lesions  in  this  general  region.  Some  of  congenital 
origin,  with  agenesis  of  the  cornu  ammonis  have  been  described. 


182         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


Anosmias  or  olfactory  agnosias  are  frequent  in  general  paresis,  and 
in  abscess  and  tumor. 


Etiology. 

I.    CONGJDNITAL  DEFECTS   OF  OLFACTORY. 


Clinical  Signs. 


II.  Senile  Involution ,  ,  . 

III.  Mechanical  Influences 

Compression  by  meningitis. 

Tearing  of  fibers;  fracture. 


IV.  Functional  Overuse 

Overstimulation;  empyema  of  sinuses  ■ 


V.  Toxic  Influences 

Influenza 

Cocaine 

Strychnine 

Nicotine. 

Alcohol 

VI.  Secondary  to  Other  Influences 
Lesion  of  V  and  VII 


>Hypei'osmia 


Psychogenic  (hysteria,  precox) .  .  . 

Fig.  69. — General  summary  of  olfactory  disturbances.     (Veraguth.) 


DISEASES  OF   THE  OPTIC   PATHWAYS  183 

The  efferent  pathways  and  synaptic  reflex  paths  of  the  olfactory 
are  many.  The  most  common  motor  reflex  is  that  of  sniffling,  with 
dilatation  of  the  nostrils.  This  is  occasionally  seen  as  the  result  of  a 
central  lesion  (dementia  precox,  paresis,  compulsion  neurosis). 
The  relation  between  odors  and  the  vomiting  reflex  is  to  be  borne 
in  mind  in  hysterical  vomiting,  furthermore  the  very  primitive  asso- 
ciations between  odor  and  sexual  complexes.  Hysterical  anosmia 
may  be  closely  associated  with  general  hysterical  hemianesthesia, 
which  latter,  however,  is  more  often  due  to  a  thalamic  lesion  than  to  an 
hysteria,  or  it  may  be  an  isolated  phenomenon  and  is  usually  classical 
of  the  mechanism  of  conversion  in  a  narrow  Freudian  sense.  The 
psychology  of  smell  and  its  complicated  relationships  to  infantile 
phantasies  of  disgust,  to  childbirth  from  intestinal  canal,  to  the 
identification  of  feces  and  money,  make  the  study  of  odor  phenomena 
of  great  import  in  the  psychoneuroses  and  psychoses.  In  certain 
psychogenic  epileptics  the  olfactory  symbolisms  are  highly  developed. 
What  relations  these  have,  either  as  cause  or  result,  to  the  cornu 
ammonis  lesions  found  in  these  epileptics  (Alzheimer)  has  not  yet 
been  determined.^  ^ 

Treatment. — The  underlying  cause  of  the  changed  olfactory  state 
needs  treatment,  not  the  state.  Local  applications  of  cocaine, 
morphine,  etc.,  are  usually  unjustified. 

DISEASES  OF  THE  OPTIC  PATHWAYS. 

The  course  of  the  light  pathways  and  its  synapses  is  extremely 
complex,  since  sight  has  become  one  of,  if  not  the  principal  tool  of 
advance  in  the  evolution  of  man's  mental  powers.  The  symptom- 
atology by  implication  of  the  pathways  is  likewise  rich  and  varied. 

The  retina,  the  mostly  decussating  optic  nerve,  ending  in  the 
external  geniculate,  the  pulvinar  of  the  thalamus  and  the  anterior 
corpora  quadrigemina,  and  finally  the  optic  radiations  ending  in  the 
calcarine  region  of  the  cortex  make  up  the  primary,  secondary  and 
tertiary  incoming  neurones  of  this  pathway.     (See  Fig.  86.) 

The  intricate  symptomatology  is  dependent  upon,  and  will  be 
discussed  in  accordance  with,  these  anatomical  divisions.  The  chief 
signs  to  be  considered  are  night  blindness,  color  blindness,  dimness  of 
vision,  blindness  in  one  or  both  eyes,  temporary  or  complete,  scotomata, 
temporary  or  permanent  hemianopsia,  hemichromatopsia,  hemiopia, 
mind  blindness  or  optical  agnosia,  photophobia,  hallucinations  and 
illusions  of  sight,  hysterical  blindness,  malingering  of  blindness, 
unilateral  or  bilateral. 

Retinitis. — Involvement  of  the  first  neurone  is  termed  a  retinitis. 
It  may  result  from  the  extension  of  an  inflammation  or  may  be  due 

'  Bailey,  P.,  Flaubert's  Epilejisy,  Proceedings  of  Charaka  Society,  New  York,  vol.  iii. 
2  Clark,  L.  P.,  The  Epilepsy  of  Dostoiewsky,  Medical  Record,  New  York,  1915. 


184 


SENSORI-MOTOR  NEUROLOGY^CRANIAL  NERVES 


Cortex 


Fig.  70. — Diagrammatic  scheme  of  optic  paths  and  chief  connections  at  four  levels. 
A,  level  of  II  and  III  nerves;  B,  level  of  IV  nerve;  C,  level  of  VI  and  VII  nerves, 
tegmentum  of  pons;  D,  spinal  cord.  Neurone  1.  Receptors  in  rods  and  cones  of  retina 
are  not  indicated  in  the  diagram.  Neurone  2.  2a,  axones  passing  to  pulvinar  of  same 
side;  2b,  axones  passing  to  corpus  quadrigeminum  same  side;  2c,  axones  passing  to 
external  geniculate  of  same  side,  all  from  temporal  side  of  retina;  from  nasal  side  2a, 
axones  crossing  in  chiasm  going  to  opposite  external  geniculate;  2/,  axones  crossing 
in  chiasm  to  go  to  opposite  anterior  corpus  quadrigeminum;  2g,  axones  crossing  in 
chiasm  to  opposite  pulvinar.  Papillomacular  bundle  fibers  partly  crossed,  partly 
uncrossed  (see  Fig.  75).  Neurone  3.  Pulvinar  axones  to  occipital  cortex;  36,  external 
geniculate  axones  to  occipital  lobes;  3e,  d,  e,  corpora  quadrigemina  fibers,  middle  layer 
decussating  (Meynert)  to  median  longitudinal  fasciculus  and  forming  tractus  tecto- 
bulbaris  et  spinalis  to  go  to  medulla  and  anterior  columns,  forming  synapses  with  third, 
fourth,  sixth,  and  seventh  nerves  and  motor  nuclei  of  spinal  nerves  (space  orientation) ; 
3/,  g,  fibers  from  interstitial  nucleus  (Cajal)  of  fasciculus  longitudinalis  posticus  forming 
part  of  longitudinal  fasciculus,  passing  to  anterior  columns,  forming  synapses  with 
III,  IV,  VI  cranial  nerves  and  motor  spinal  nerves.  Neurone  4.  Axones  from  oculo- 
motor, facial,  and  spinal  nuclei.     (Strong.) 


DISEASES  OF   THE  OPTIC   PATHWAYS  185 

to  toxic  or  to  hidden  constitutional  factors.  The  chief  indications  are 
ocular  discomfort  or  photophobia,  diminution  of  the  visual  acuity, 
appearance  of  scotomata,  general  contraction  of  the  visual  fields, 
micropsia,  megalopsia  or  metamorphopsia. 

Different  grades  of  retinitis  are  distinguished  ophthalmoscopically. 
The  chief  types  are  simple,  albuminuric,  syphilitic,  diabetic,  hemor- 
rhagic and  anemic  retinitis. 

In  simple  retinitis  there  is  clouding  particularly  of  the  superficial 
layers,  in  patches  or  in  larger  portions  at  the  posterior  pole.  The 
veins  are  dull  and  dark  and  full,  and  seem  imbedded  in  the  swollen 
or  hazy  retina.  Sight  is  dim  and  worse  in  spots  (scotomata).  The 
disorder  usually  involves  first  one  eye  and  then  the  other. 

Albuminuric  retinitis  is  frequent  in  Brights  (25-40  per  cent.). 
Headache  and  loss  of  vision  in  a  middle  aged  to  older  person  are 
the  usual  signs.  There  are  characteristic  changes  in  the  retina  and 
albumin  and  casts  in  the  urine.  Cirrhotic  kidney  is  the  most  fre- 
quent lesion.  The  chief  change  is  an  arteriosclerosis  of  the  retinal 
vessels.  They  are  unduly  tortuous  and  show  contractions  and  widen- 
ings,  often  being  beaded.  There  is  also  a  translucency  in  the  retina, 
white  stripes  accompany  the  vessels.  The  veins  are  likewise  tortuous, 
and  disturbances  of  circulation  show  particularly  at  venous-arterial 
crossings.  Retinal  edema  with  grayish  opacity  shows.  Hemorrhages 
are  frequent.  The  margins  of  the  disk  become  obscured,  the  nerve 
expanding  into  the  retina  without  sharp  lines  of  demarcation.  The 
disk  may  be  much  swollen,  wooley  in  appearance,  and  much  extrav- 
asated.     Fatty  degenerations  with  "snow  bank"  appearances  occur. 

Blindness,  scotomata,  dimness  of  vision  appear  as  in  simple  neuritis, 
but  chronic  cases  of  albuminuric  retinitis  may  be  present  with  little 
loss  of  visual  acuity  in  the  early  stages.  Permanent  impairment  of 
vision  is  the  rule.  Albuminuric  retinitis  accompanied  by  hemorrhages, 
and  fatty  degeneration  of  the  retina,  in  a  patient  over  35  to  40  usually 
portends  a  fatal  issue  within  comparatively  few  years. 

Syphilitic  retinitis  is  probably  much  more  frequent  than  has  been 
supposed.  It  may  result  from  hereditary  or  acquired  syphilis,  in  the 
latter  case  appearing  soon  after  infection.  Clinically  there  is  contrac- 
tion of  visual  fields,  dimness  of  vision,  may  be  night  blindness,  or  marked 
dimness  of  vision  with  poor  illumination.  Shimmering  lights  which 
are  persistent  and  annoying  occur,  with  micropsia  and  at  times  meta- 
morphopsia. Central,  partial  or  complete  scotomata  are  fairly 
constant. 

The  ophthalmoscope  shows  hyperemia  with  serous  exudation  much 
resembling  the  albuminuric  variety"  but  in  milder  degree.  Hemor- 
rhages are  much  rarer,  and  the  "snow  bank"  glistenings  much 
less  pronounced.  Opacity  about  the  disk  is  a  variant  feature,  with 
inflammation  of  the  uveal  tract. 

Hemorrhagic  retinitis  is  of  import,  but  must  be  referred  to  works 
on  ophthalmology  with  the  other  types. 


186 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


Retinitis  pigmentosa  is  an  hereditary  variety  of  primary  retinal 
degeneration  showing  night  blindness  with  striking  frequency.  Nettle- 
ship's  famous  study  of  a  French  family  showed  this  to  be  a  striking 
instance  of  Mendelian  dominance.     (Plate  VI.) 

Optic  Nerve.  —  The  disorders  affecting  the  second  optic  neurons 
fall  into  two  groups:  those  affecting  (a)  the  optic  nerve,  (h)  its  ter- 
minations in  the  midbrain  structures. 

(a)  Diseases  of  the  Optic  Nerve. — Here  three  situations  need  to  be 
distinguished:  (1)  whether  the  affection  lies  anterior  to  the  chiasm, 
(2)  whether  it  involves  the  chiasm,  or  (3)  lies  behind  the  chiasm  in  the 
path  of  the  second  optic  neurone. 


,^v^V 


,0^ 


Fig.  71. — Scheme  of  papillomacular  bundle.     (Wilbrand  and  Sanger.) 


(1)  Diseases  of  the  Optic  Nerve  before  reaching  the  chiasm:  Optic 
Neuritis,  in  general  sense.  Three  types  are  distinguishable  with 
pronounced  symptomatology.  They  are:  (a)  Axial  Neuritis,  (b) 
Interstitial  Peripheral  Neuritis,  and  (c)  Diffuse  Neuritis. 

A.  Axial  Neuritis. — ^This  is  a  system-disease  of  the  papillomacular 
bundle,  involving  the  nerve  in  front  of  the  chiasm.  It  may  be  acute 
or  chronic. 

Acute  Axial  Neuritis.— Occurs  usually  in  young  adults,  12  to  24, 
more  particularly  women.  There  appears  sudden  clouding  or  dimness 
of  vision,  and  occasional  photopsias.  A  frontal  or  temporal  headache, 
or  deep  pain  in  the  orbit,  made  worse  by  pressure  or  movement  of  the 
eyeballs  is  present.  The  loss  of  sight  is  rapid,  reaching  a  maximum 
usually  in  five  days,  and  often  is  so  severe  that  the  patient  can 


I 

II 

III        i 

N     f^ 


1 


V  ^ 


vn 


T 


"1 


I 

n 

IE 


V 


o  u  5 


iffisismAR      2522  3 
VliSJJJ|2  4  19    ^37!      463  2  4    p3  4^^.2ifl§S^M 


25^2     3  36433516      SSS^S     2574 


vni     i 

DC 
X 


9    3  35  4ii,6S      47       5 

I 

O    4  24 

I 
6 


SVID 


Descent  of  a  Fd  Nettleship,  Based  on  Cunier's  Records  with  Later 
Additions.)  Detail.  The  Affected  Man  (DR)  Who  Married  the 
Affected  ^A/"cheration  VII. 

Black  symbols  show  to  a  dominant  factor.  According  to  the  records  there  is  a  great 
excess  of  normas,  the  table  gives  a  remarkable  illustration  of  the  permanence  and 
mode  of  descent 


Descent  of  a  Form  of  Stationary  Night  Blindness.      (Condensed  from  the  Chart  Published  by  Nettleship,   Based  on  Cunier's   Records  with    Later 

Additions.)       Only  those  Families  Which  Contain  Affected  Members  are  Here  Set  Out  in  Detail.      The    Affected    Man   (DR)   Who  Married  the 

Affected  Woman  (DR)  is  Represented  by  the  Sixteenth  Black  Symbol  from  the  Left  in  Generation  VIl. 
Black  symbols  show  the  night-blind  individuals.     The  descent  is  always  through  the  affected,  showing  that  the  condition  is  due  to  a  dominant  factor.        According    to    the    records    there   is   a    greai 

excess  of  normals  over  the  affected.        It  cannot  be  said  that  the    responsible  factor  is  a  simple  allelomorph.       Nevertheless,    the    table    gives    a    remarkable    illustration    of    the  permanence  and 

mode  of  descent  of  a  dominant  variety.       (Bateson.) 


DISEASES  OF  THE  OPTIC  PATHWAYS 


187 


just  count  fingers  at  15  feet,  or  is  blind.  With  the  loss  of  sight  the 
lieadache  lets  up.  There  may  be  no  retinal  changes.  The  pupil  of 
the  affected  eye  is  larger,  and  is  usually  sluggish  to  direct  light 
stimulation,  but  shows  no  consensual  light  reflex  loss. 

After  the  acute  stage  is  over  there  is  gradual  recovery  of  the  sight 
in  the  periphery  with  various  degrees  of  persistent  central  scotomata. 
The  loss  may  be  unilateral  or  bilateral  and  absolute,  or  unilateral  or 
bilateral  to  color  only,  or  various  gradations  of  these  paracentral 
scotomata,  etc.  The  scotomata  gradually  diminish  and  after  six  to 
eight  weeks,  with  proper  therapy,  may  entirely  disappear  (Fig.  72). 

The  fundus  picture  may  remain  normal  throughout  or  show  a 
papillitis.  This  will  depend  upon  how  far  back  of  the  optic  disk  the 
lesion,  which  is  usually  a  vascular  one,  occurred.  When  there  is  a 
papillitis  it  shows  slight  paling  of  the  temporal  half  or  halves  of  the 
fundi  (Fig.  72). 

Etiology. — The  most  frequently  ascribed  cause  is  exposure  to  cold. 
This  is  probably  only  an  incident  to  other  real  causes  such  as  infec- 


FiG.  72. — Central  scotomata  in  acute  axial  neuritis.     (Wilbrand  and  Sanger.) 

tious  disease — syphilis,  tuberculosis,  typhoid,  erysipelas,  sinusitis, 
influenza,  mumps,  pneumonia,  tonsillitis,  cerebrospinal  meningitis, 
malaria,  beri  beri,  etc.,  or  toxemias,  such  as  those  of  pregnancy, 
nephritis,  burns,  CO  poisoning,  methyl  alcohol,  quinine,  filix  mas, 
morphine,  etc. 

Treatment. — Hot  baths,  and  treatment  of  cause,  as  antisyphilitic  in 
syphilis,  etc. 

The  Chronic  Form  is  much  more  frequent.  It  is  the  classical  situa- 
tion in  chronic  alcohol  or  nicotine  poisoning,  and  affects  males  more 
often.  Here  the  course  is  a  chronic  one,  spreading  over  several 
months  or  years.  The  blindness  appears  slowly,  and  at  first  consists 
of  a  central  scotoma  for  colors,  or  of  a  hemeralopia,  the  patient 
seeing  better  in  the  dusk  than  in  the  bright  light  (fatigue).  The 
scotomata  become  more  marked  if  the  poisoning  continues  (Fig.  72). 

The  type  of  scotoma  varies  widely.  Bilateral,  fairly  symmetrical, 
oval  scotomata  for  red  and  green,  lying  between  the  blind  spot  and 
the  fixation  point  is  the  early  picture.     It  usually  starts  as  a  defect 


188 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


for  red,  stretching  toward  the  bhnd  spot  (Fig.  73).  The  chief  defect 
usually  lies  about  2°  to  8°  from  the  fixation  point.  Absolute  central 
scotomata  are  rare. 

Vision  is  usually  diminished,  and  more  on  one  side  than  the  other. 
In  monocular  reading  the  type  to  the  right  of  the  fixation  point  is 
not  clear  for  the  right  eye,  while  for  the  left  eye  the  defect  lies  to  the 


Fig.  73. — Scotoma  for  red  and  green  in  tobacco  axial  neuritis.    (Wilbrand  and  Sanger.) 

left.  The  defect  in  vision  bears  little  direct  relation  to  the  size  of  the 
scotomata.  Pupillary  anomalies,  diminution  of  both  light  and  accom- 
modation reflexes  and  pseudotabetic  pictures  are  to  be  found. 

The  fundus  picture  may  be  normal  with  gross  defect  in  vision  and 
large  scotomata,  or  there  may  be  hyperemia,  a  mild  neuritis,  with 
some  temporal  pallor.  If  marked  pallor  is  present  it  speaks  in  general 
for  a  more  severe  process. 


Fig.  74. — Beginning  scotoma  for  red  at  the  onset  of  a  tobacco  or  alcoholic  axial  neuritis. 

(Wilbrand  and  Sanger.) 

The  course  is  chronic.  Removal  of  the  cause  may  save  the  sight,  but 
slight  scotomata  may  persist.    Atrophy  is  the  end  in  the  severe  cases. 

Pathology. — The  changes  in  both  acute  and  chronic  types  occur  in 
the  papillomacular  bundle.  They  are  degenerative  rather  than 
inflammatory  changes,  with  greatest  severity  in  the  optic  canal, 
because  of  the  richer  vascular  supply  of  the  nerve  trunk  at  this  point. 


PLATE    Vri 


Fig.    1 


Normal  Eye-ground  (average  tint).     (Norris  and  Oliver.) 


Fig.   2 


Ophthalmoscopic  Appearances  in  Early  Stage  of  Optic  Neuritis. 

(Norris  and   Oliver.) 


DISEASES  OF   THE  OPTIC  PATHWAYS 


189 


Etiology. — The  chief  causes  are  alcohol  and  tobacco.  Other  less 
frequent  poisonings  are  by  carbon  bisulphide,  in  the  rubber  indus- 
tries, arsenic,  lead,  sulphuric  acid, 
anemia  (anchylostoma),  diabetes, 
aniline,  gasoline. 

Hereditary  Forms. — These  oc- 
cupy intermediary  positions.  One 
type  affects  usually  the  males, 
passing  through  unaffected  females 
(Knight's  move  in  heredity) .  These 
develop  acute  or  subacute  head- 
aches about  the  age  of  20,  with 
gradually  diminishing  vision.  Un- 
equal bilateral  scotomata  appear 
and  gradually  increasing  central 
blindness.  There  is  usually  a 
subacute  and  progressive  stage  for 
about  six  months,  one  eye  lagging 
behind  the  other  in  its  deterioration. 

Gradually  increasing  central  sco- 
tomata show.  Nyctalopia  is  fre- 
quent. The  marginal  vision  is 
retained,  save  for  colors.  Total 
blindness  develops  in  about  two- 
thirds  of  the  cases.  One-third 
remain  more  or  less  stationary 
or  even  improve.  In  mild  cases 
central  color  scotomata  alone  ap- 
pear, or  there  is  diffuse  loss  of 
color  sense  without  scotomata. 

The  fundus  picture  is  variable, 
but  the  presence  of  atrophic 
changes,  usually  temporal,  stands 
in  marked  contrast  with  any  other 
changes  in  the  nervous  system. 

Hereditary  syphilis  plays  a  role 
in  some  cases.  In  others  there  is 
an  anomaly  in  the  germ  plasm. 

B.  Interstitial  Peripheral  Neuritis. 
— Here  the  disease  involves  the 
periphery  of  the  optic  nerve  rather 
than  the  central  or  eccentrically 
lying  papillomacular  bundle.  Con- 
centric limitation  of  the  field  of 
vision,  for  white  and  colors,  is  the 
chief  finding  rather  than  central  scotomata.  Here  there  is  a  peripheral 
inflammation  of  the  nerve  trunk,  starting  in  the  pia  and  proceeding 
inward  in  the  septa. 


Fig.  75. — Course  of  the  papillomacular 
bundle  at  different  points  in  the  optic 
tract.     (Wilbrand  and  Sanger.) 


190 


SENSORI-MOTOB  NEUROLOGY— CRANIAL  NERVES 


The  concentric  limitation  of  vision  is  rarely  observed  in  the  beginning. 
As  it  slowly  advances  the  patients  become  uncertain  of  space 
localization  and  need  to  turn  the  eyes  frequently  to  get  clear  pictures 
of  where  they  are  going.     Central  vision  is  usually  sharp  even  for  color. 

The  fundi  show  simple  or  neuritic  atrophy,  occasionally  choked 
disks.     Very  variable  fields  are  observed  (Figs.  75,  76,  77,  78). 

Hysterical  limitation  of  the  field  is  to  be  considered  here.  Normal 
fundi  are  consistent  with  a  true  interstitial  neuritis,  since  lesions  lying 
far  back  in  the  nerve  trunk  may  cause  little  or  no  disk  changes.  A 
psychoanalytic  anamnesis  usually  will  clear  up  a  possible  hysteria. 
Tabes  with  neuritis  may  begin  as  an  interstitial  neuritis.  The  cy to- 
biological  findings  will  clear  up  the  diagnosis. 

Etiology. — Syphilitic  meningitis  of  the  base  is  the  most  frequent 
cause.  A  negative  Wassermann  is  not  a  just  criterion  to  deny  specific 
medication.     Other  etiological  factors  are  measles,  diphtheria,  influ- 


FiG.  76. — Visual  fields  in  a  patient  with  hereditary  axial  neuritis.     Fields  for  white 

normal,  for  blue ,  and  for  red  .  .  .  .,  concentrically  contracted.     Absolute  central 

scotoma  with  larger  bordering  scotoma  for  blue  and  red.     (Wilbrand  and  Sanger.) 


enza,  myelitis,  gonorrhea,  sinusitis,  typhoid,  lead,  diabetes,  lepto- 
meningitis, cerebrospinal  and  tuberculous  meningitis. 

The  therapy  is  causal,  usually  specific.  Salvarsan  is  less  to  be  feared 
than  an  active  syphilis.  Most  so-called  neurorecidives  causing  blind- 
ness are  due  more  to  the  syphilis  than  to  the  arsenic. 

C.  DifEuse  Neuritis. — Here  the  inflammatory  process  involves  the 
entire  nerve  stem  resulting  in  marked  amblyopia,  or  blindness.  It 
may  be  implicated  locally  or  throughout  its  entire  course  from  the 
retina  to  the  chiasm.  In  acute  myelitis  this  severe  inflammation  at 
times  occurs.  Infectious  diseases  are  important.  Influenza  here 
plays  almost  as  large  a  role  as  syphilis. 

The  infections  and  toxemias  mentioned  in  the  preceding  paragraphs 
may  also  induce  a  total  optic  neuritis.  Malaria,  scarlet  fever,  yellowl 
fever,  erysipelas  may  be  added  to  the  causes.  Orbital  sinus  disease^ 
is  important,  and  also  multiple  sclerosis  (Fig.  81). 

Other  atrophic  states,  double,  one  sided,  total  or  partial,  occur, 


DISEASES  OF  THE  OPTIC  PATHWAYS 


191 


I.  R. 

Fig.  80 
Figs.  77,  78,  79,  and  80. — Visual  fields  in  cases  of  interstitial  peripheral  optic  neuritis 
from  syphilis. '  (Wilbrand  and  Sanger.) 


192 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


either  in  the  papillae,  from  pressure  of  a  glaucoma,  or  may  be  descending 
atrophies  from  higher  lying  causes  such  as  brain  tumor,  hydrocephalus. 
Primary  progressive  atrophy,   arising  by  itself,  probably  does  not 


Fig.  81. — ^ Visual  field  with  diffuse  neuritis  due  to  multiple  sclerosis.  On  right  side  a 
temporal  hemianopic  color  scotoma,  on  the  left  intermittently  appearing  hemianopic 
uncertainty  scotoma.      (Wilbrand  and  Sanger.) 


Fig.  82. — Visual  field  showing  atrophy  in  tabes.     Field  for  blue 
red  -  -  -  -  .      (Wilbrand  and  Sanger.) 


,  for 


Fig.  83. — Optic  atrophy  in  tabes  with  loss  of  color  discrimination. 
(Wilbrand  and  Sanger.) 


exist.  The  most  suggestive  cause  of  an  isolated,  bilateral,  progressive 
optic  atrophy  without  other  tangible  neurological  signs  is  tabes. 
Cytobiological  tests  wiU  complete  the  diagnosis  (Figs.  82  and  83). 


DISEASES  OF   THE  OPTIC  PATHWAYS 


193 


Disease  at  or  about  the  Chiasm. — The  anatomical  peculiarities,  due 
to  the  crossing  of  the  fibers  at  the  chiasm,  introduces  certain  definite 
signs  which  are  of  value.  Scotomata  and  concentric  limitation  are 
replaced  by  hemianopsias  of  varying  type. 

In  lesions  in  front  of  the  chiasm  bitemporal  hemianopsia  will  be 
present.  This  is  rare.  A  lesion  behind  the  chiasm,  usually  in  the 
sella  turcica,  and  not  infrequent,  as  in  pituitary  disease,  causes  a 
binasal  hemianopsia,  partial  or  complete.  Lesions  to  the  right  or 
left  of  the  chiasm  will  cause  incomplete  homonymous  hemianopsias — 
whereas  lesions  in  the  tract  back  of  the  chiasm — i.  e.,  in  the  midbrain 
or  optic  radiations  or  occipital  lobes  will  cause  a  usuall}^  more  complete 
homonymous  hemianopsia. 

Horizontal  hemianopsias,  either  superior  or  inferior,  occur  in 
chiasm   lesions   from   pressure   above   or   below.     They   are  readily 


75         °p        l"^^        720 
60  \  I  l_         /  135 


SOO  ssS 

Fig.  84. — Quadrant  hemianopsia  of  lower  right  segment  due  to  hemorrhagic  destruction 

of  the  external  geniculate. 


explained  from  the  position  of  the  crossing  fibers  in  the  chiasm.     Such 
hemianopsias  may  rarely  occur  from  retinal  causes. 

A  common  cause  for  chiasm  changes  is  hypophyseal  tumor. 
Syphilis,  however,  is  specially  frequent  in  just  this  situation,  most 
basal  syphilitic  meningitic  processes  beginning  here.  The  process 
spreading  forward  to  the  optic  stem  produces  a  multiplicity  of  field 
changes.  Thus  one  may  have  partial  scotomata,  monocular  temporal 
hemianopsia,  bitemporal  hemianopsia  (the  most  frequent),  temporal 
hemianopsia  with  blindness  of  one  eye,  blindness  in  one  eye  and  nasal 
hemianopsia  of  the  other,  blindness  in  both  eyes.  This  very  great 
irregularity  and  changeability,  advancing  or  receding  under  treatment, 
is  of  much  importance  in  excluding  a  hypophyseal  tumor.  A  loss  of 
the  hemianopic  pupillary  reaction  is  of  importance  in  making  a 
definite  localizing  diagnosis. 

The  papillary  changes  are  variable.     Other  signs  of  basal  syphilitic 
meningitis  are  discussed  in  the  chapter  on  Cerebral  Syphilis  (q.  v.). 
1.3 


194 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


Affections  of  the  chiasm  are  more  rarely  encountered  as  a  result  of 
trauma,  brain  tumor  with  general  pressure,  cavernous  sinus  disease, 
cerebrospinal  and  tuberculous  meningitis,  bone  disease,  aneurism  and 
arteriosclerosis  of  the  carotids. 

Thalamus  Disease. — In  lesions  of  the  optic  tract  posterior  to  the 
pulvinar,  i.  e.,  in  the  third  optic  neurone,  pupillary  disturbances  are 
absent.  Thus  Wernicke  has  shown  that  by  careful  illumination  of  the 
blind  side  of  the  eye  one  can  distinguish  between  an  hemianopsia  in 
the  second  optic  neurone  (by  loss  of  pupillary  light  reflex)  and  a  hemi- 
anopsia of  the  third  optic  neurone  (intact  hemiopic  pupillary  reflex). 
As  a  matter  of  fact  this  test  is  extremely  difficult  to  perform,  but 
fortunately   lesions   in   the   midbrain — end  station   of  second   optic 


C  geiv.  cxt. 


Fig.  8.5. — Site  of  lesion  in  external  geniculate  giving  rise  to  quadrant  hemianopsia 
seen  in  Fig.  84  and  the  secondary  degenerations  in  Fig.  86.  C.  gen.  ext.,  external  genic- 
ulate; h(B7n,  hemorrhage;  C.i.,  internal  capsule;  F.S.,  fissure  of  Sylvius;  S.8.,  optic 
radiations. 


neurone — are  almost  invariably  accompanied  by  other  sensory  signs 
(usually  a  complete  or  incomplete  thalamic  syndrome  (g.  v.),  thus 
aiding  in  the  diagnosis. 

The  distributions  of  the  second  optic  neurone  are  multiform.  Some 
fibers  end  in  the  corpora  quadrigemina.  Their  implication  causes 
pupillary  changes;  others  end  in  the  thalamus  (pulvinar),  and  their 
involvement  causes  no  disturbances  of  vision.  The  majority  of  the 
fibers  end  in  the  external  geniculate  bodies.  These  are  in  close 
relation  to  the  auditory  tract,  the  sensory  tracts,  the  oculomotor 
nuclei  and  pyramidal  tracts.  Hence  lesions  of  the  optic  tract  in  the 
geniculate  region  cause  not  only  homonymous  hemianopsias,  often 


DISEASES  OF   THE  OPTIC  PATHWAYS 


195 


only  quadrant,  but  they  are  also  liable  to  be  complicated  by  the 
involvement  of  these  other  near  lying  structures.  Isolated,  quadrant 
hemianopsias  may  mean  small  hemorrhages,  thrombi,  emboli,  tumors, 
or  encephalitis  in  the  external  geniculate  (poliomyelitis — rare)  as  well 
as  from  lesions  farther  back  in  the  tractus  (Figs.  84,  85,  and  86). 

Cortex  Disease.- — Lesions  of  the  end  stations  of  the  optic  tract  or  its 
associated  areas  in  the  occipital  lobe  may  cause  mind  blindness,  i.  e., 
optic  agnosia.  Here  the  patient  may  have  no  disturbance  of  sight, 
or  he  may  have  partial  hemianopsias,  but  is  unable  to  recognize  words 
or  objects  previously  known,  speech  being  intact. 


Cu/i. 


Fig.  86. — Showing  atrophic  degenerations  in  optic  radiations  (atr.)  from  hemorrhage 
in  external  geniculate  (Fig.  85),  giving  rise  to  quadrant  hemianopsia  of  Fig.  84.  Cun., 
cuneus;  Calc,  calcarine  fissure;  f.l.i.,  inferior  longitudinal  fasciculus;  I,  left  hemisphere; 
atr.,  atrophy.     (Henschen.) 


Optic  hallucinations  are  present  in  disorder  of  the  optical  end 
stations  in  the  occipital  lobe.  When  they  show  definite  projections 
in  space  one  can  make  an  accurate  localization  of  the  portion  of  the 
lobe  involved.  This  may  be  of  great  value  in  determining  the  site 
of  a  tumor  or  abscess  formation. 

The  chief  arterial  supply  of  the  posterior  neurones  is  drawn  from 
the  calcarine  branch  of  the  posterior  cerebral.  The  most  occipital 
portion  is  supplied  by  the  median  cerebral.  The  anterior  cerebral 
sends  branches  which  innervate  the  optic  radiations  just  posterior 
to  the  corpus  callosum,  but  lesions  of  this  artery  at  this  place  cause  no 
definitely  recognizable  lesions. 


196 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


lit 


S  g  ca  oj 

^•^  |--S'g  o  8  8 


DISEASES  OF  THE  OCULOMOTOR  NERVES  197 

The  cortical  disturbances  of  vision  from  lesions  of  the  temporal 
or  occi})ital  lol)es  cannot  be  entered  into  more  fully  here.  See  chapters 
on  Aphasia,  Syphilis  of  the  Brahi  (Paresis),  Brain  Tumor,  Hemiplegia, 
Thrombosis,  Arteriosclerosis,  etc.^ 


DISEASES  OF  THE  OCULOMOTOR  NERVES. 

Ocular  Nerves :  Third,  Fourth,  Sixth. — Disorders  of  the  functions 
of  these  nerves  are  best  discussed  under  a  general  head,  since  the  usual 
ocular  palsies  are  often  complex  syndromes  in  which  one  or  more  of 
these  nerves  are  involved. 

The  third  nerve  is  a  motor  nerve  for  all  of  the  muscles  of  the  eye- 
ball, save  the  external  rectus,  and  the  superior  oblique,  which  latter 
receive  their  motor  fibers  from  the  sixth  and  fourth  nerves  respectively. 
The  third  nerve  also  supplies  the  levator  palpebrse,  the  ciliary  muscle 
and  the  contracting  fibers  of  the  pupil.  The  dilating  fibers  of  the 
pupil  receive  a  branch  from  the  sympathetic.  Deep  sensibility  fibers 
also  pass  in  the  motor  roots     (Sherrington)  .^ 

Third  Nerve  Palsies. — These  are  often  very  complicated  and  may 
be  central  or  peripheral,  complete  or  partial.  Complete  paralysis 
of  both  third  nerves  is  rare,  partial  palsies  are  the  rule.  Unilateral 
palsy  of  all  of  the  external  muscles  governed  by  the  third  nerve  (often 
termed  ophthalmoplegia  externa)  is  due  usually  only  to  a  lesion 
involving  the  second  or  peripheral  motor  neurones  of  the  third  nerve. 
Bilateral  external  ophthalmoplegia  may  occur  also  from  lesions  of  the 
mesencephalon  and  cortical  oculomotor  paths.  The  chief  causes  for 
central  palsies  are  various  types  of  encephalitis,  polioencephalitis,  either 
infectious  as  in  Heine-Medin's  disease,  or  toxic  as  in  alcoholism. 
(Wernicke's  polioencephalitis  superior.)  Syphilitic  thrombosis  may 
cut  off  the  blood  supply  of  the  nuclei.  Pressure  from  the  aqueduct 
above,  or  third  ventricle  may  cause  pressure  palsies,  usually  of  irregular 
distribution.     (Nothnagel's  Syndrome.     Pineal  Syndrome.) 

Peripheral  palsies  are  more  frequently  due  to  disease  at  the  base, 
usually  basal  syphilitic  meningitis,  tumor,  tuberculosis,  hemorrhage, 
traumas  (rarely)  or  are  occasioned  by  involvement  of  the  fibers  as  they 
pass  through  and  about  the  red  nucleus  by  tumor,  multiple  sclerosis, 
or  when  implicated  in  a  thrombotic  or  hemorrhagic  softening  of 
the  cerebral  peduncle — Millard-Gubler,  Benedict,  Fovilles'  syn- 
dromes, red  nucleus  syndromes.  Infectious  disease  neuritis  may  also 
occasion  peripheral  palsies.  Pressure  from  aneurism  of  the  internal 
carotid,  and  thrombosis  of  the  cerebral  sinuses  (sinus  cavernosus)  may 
also  cause  peripheral  palsies.  A  special  herpes  zoster  ophthalmicus 
is  known.     Exophthalmic    goitre  and   diabetes   are   special   causes. 


'  See  Wilbrand  and  Sanger,  and  Henschen  in  Lewandowsky  Handbuch,  vol.  iii. 
2  Sherrington  and  Tozer,  Proc.  Royal  Soc,  1910. 


198         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


H.giUet: 


Fig.  88. — Fovilles'  syndromes,  with  anterior  and  posterior  pontine  syndromes. 
Hemiplegia,  cerebral  type,  with  (a)  conjugate  deviation  of  the  head  and  eyes,  (6)  by 
lesions  of  the  upper  portion  of  the  pons,  right  side,  involving  the  anterior  portion  of 
the  pons  and  the  region  of  the  tegmentum.  On  the  left  side  there  is  a  contralateral  hemi- 
plegia of  the  limbs,  of  the  lower  part  of  the  face  and  of  the  tongue,  because  of  the  involve- 
ment of  the  pontine  pyramidal  fibers  Py  (cortico  spinal  pyramidal  fibers,  cortico  nuclear 
facial  and  lymphoglossal  fibers.  In  c,  right-hand  figure,  there  is  a  single  lesion  which  in- 
volves the  tegmentum  at  its  antero-internal  angle  and  destroys  the  head-turning  (cepha- 
logyric)  and  eye  turning  (oculogyric)  fibers  of  the  right  side  which  at  this  level  are  situated 
in  the  pes  lemniscus  and  the  internal  portion  of  the  median  fillet  giving  rise  to  conjugate 
deviation  of  the  head  and  of  the  eyes.  By  reason  of  the  predominant  abtion  of  the  antago- 
nists the  head  is  inclined  to  the  right  and  the  eyes  look  to  the  right,  the  patient  looks  to  the 
side  of  the  lesion.  In  a  there  are  multiple  isolated  lesions.  Four  large  foci  in  the  anterior 
portion  destroy  the  pontine  pyramidal  fibers  with  a  resulting  crossed  contralateral  hemi- 
plegia of  the  extremities,  the  face,  and  the  tongue.  Another  focus  occupies  the  posterior 
internal  portion  of  the  tegmentum  and  destroys  the  internuclear  oculogyric  fibers  of 
the  posterior  longitudinal  bundle  which  directly  unites  the  nuclei  of  the  sixth  and  third 
nerves  and  vice  versa.  There  results  a  paralysis  of  the  eyeballs  by  which  they  cannot 
turn  sideways  toward  the  right — right  oculorotary  paralysis — by  reason  of  the  predom- 
inance of  the  antagonists  the  patient  looks  to  the  left.  The  patient  looks  away  from  the 
lesion  toward  the  paralyzed  members.  The  cortical  oculorotary  fibers  and  the  pes  lem- 
niscus are  intact.  For  details  of  structure  and  abbreviations,  see  chapter  on  Midbrain 
Lesions.     (Dejerine.) 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


m 


Fig.  89. — Pontine  syndrome,  with  eye  palsies  of  central  origin  and  sjTingomyelic  dis- 
sociation. There  is  here  a  crossed  hemianesthesia  with  alternating  paralysis  of  the 
VI  and  VII  cranial  nerves,  anesthesia  of  the  V  nerve  due  to  hemorrhage  in  the  lateral 
and  lower  portion  of  the  pontine  tegmentum  of  the  left  side.  The  right-hand  figure 
shows  the  hemianesthesia,  dissociated  as  in  syringomyelia  (hemianalgesia  and  hemi- 
thermanesthesia  due  to  lesion  of  the  crossed  sensory  pathways  of  the  lateral  portion  of 
the  reticular  formation.  There  is  preservation  of  the  tactile  and  postural  sensibilities 
and  of  the  stereognostic  sense,  because  of  the  incomplete  extension  of  the  lesion  to  the 
median  lemniscus  (Rm).  The  left-hand  figure  shows  (1)  atrophic  paralysis  of  the  VII 
nerve  with  reaction  of  degeneration,  lagophthalmia,  drooping  of  the  lips,  loss  of  facial 
mimicry,  paralysis  of  the  entire  left  facial  (VII)  indicated  (Fig.  a) ;  (2)  anesthesia  of 
the  face,  following  involvement  of.  the  descending  root  of  the  trigeminus  (see  V  on  a^) ; 
(3)  paralysis  of  the  external  rectus  with  convergent  strabismus  by  reason  of  the  over- 
action  of  the  antagonists.  Furthermore,  there  is  a  paralysis  of  the  lateral  movements 
of  the  eyeballs  toward  the  left  notwithstanding  the  integrity  of  the  posterior  longitu- 
dinal fasciculus  (Ftp.),  of  the  nucleus  of  the  VI  and  of  the  adjacent  reticular  formation. 
The  lesion  of  Deiters'  nucleus,  and  of  the  labyrinthine  oculorotary  fibers  which  unite 
Deiters'  nucleus  (ND)  to  the  nuclei  of  the  III  and  VI  causes  this.  By  reason  of  the  over- 
action  of  the  antagonists  the  patient  looks  to  the  right.  (After  Dejerine.)  For  abbre- 
viations of  the  anatomical  sketch  see  section  on  Midbrain. 


200 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


Transitory  third  nerve  palsies  occur  in  the  ch'sorder  known  as  ophthal- 
moplegic migraine  (q.  v.). 

Syndromes. — The  compound  character  of  the  nuclei  and  the  loosely 
arranged  bundles  making  up  the  nerve  explain  the  great  range  in 
symptomatology.  Obersteiner  (5th  edition,  1912)  follows  Bernheimer 
chiefly  in  his  teaching  regarding  the  complicated  question  of  the  locali- 
zation of  the  brain  stem  nuclei.^  Thus  it  will  be  seen  that  from  before 
backwards  the  nuclei  are  arranged  as  follows :  Levator  palpebrse,  rectus 
superior,  rectus  inferior,  obliquus  inferior,  rectus  inferior,  trochlearis. 

A  complete  unilateral  palsy,  probably  nuclear  (ophthalmoplegia 
completa),  would  then  cause  ptosis,  wrinkling  of  forehead  on  same 


I    u  I 


Fig.  90. — Scheme  of  oculomotor  nuclei  modified  fromi  Bernheimer.  Basal  projec- 
tion,  ikf,  median  nucleus;  S.'PF'.,  Edinger  Westphal  sympathetic  nucleus.     (Obersteiner.) 

side,  wide  pupil,  irresponsive  to  light  and  accommodation,  eye  turned 
outward  and  slightly  downward.  Double  vision  is  present  and  some 
dizziness  in  the  early  stages.  A  variety  of  individual  rhuscle  palsies 
may  also  result  from  either  nuclear  or  peripheral  involvement  as 
indicated — ophthalmoplegia  externa,  when  the  pupil  is  not  involved; 
ophthalmoplegia  interna  when  only  the  internal  muscles  are  involved — 
a  rare  condition. 

The  distinction  of  nuclear  from  peripheral  palsies  is  usually  made 
on  the  basis  of  accompanying  symptoms — sensory  or  motor,  due 
to  implication  of  the  red  nucleus,  or  of  the  cerebral  peduncles.     In 


See  Kidd,  Rev.  Ntu.  and  Psych.,  xi,  507. 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


201 


the  absence  of  these  accessory  symptoms  (Weber-Giibler,  Benedict 
syndromes,  rubrospinal  syndrome)  the  distinction  may  be  impossible. 
There  is  no  single  disease  process  to  which  the  term  ophthalmoplegia 
may  be  rigidly  applied.  Hence  there  is  no  general  course  and  no 
general  treatment.  The  various  palsies  must  be  interpreted  on  the 
basis  of  the  dynamic  factors,  and  the  treatment  must  be  founded 
upon  the  causation.  Syphilis  is  responsible  for  the  majority  of  these 
palsies,  and  calls  for  verification  by  the  cytobiological  tests  and 
prompt  antisyphilitic  treatment,  best  by  salvarsan  and  hypodermic 
injection  of  mercury.  (See  chapter  on  Syphilis  of  the  Nervous 
System.) 

Isolated  involvement  of  the  pupillary  apparatus  may  be  discussed 
here.     Contracted  pupils,  irregular  pupils,   unequal  pupils,  dilated 


H. 


I      Tr. 


Fig.  91. — Scheme  of  oculomotor  nuclei,  modified  after  Bernheimer.    Sagittal  projec- 
tion.   Lettering  as  before.     (Obersteiner.) 


pupils,  etc.,  have  been  discussed  (see  Symptomatology).  A  dilated 
pupil  with  loss  of  accommodation  reflex  is  frequently  seen  in  severe 
alcoholism  (Korsakow's  syndrome).  It  may  be  present  also  in  optic 
nerve  disease — combined  with  loss  of  light  reflex  as  well.  A  loss  of 
light  reflex  with  preservation  of  the  accommodation  reflex  (reflex 
iridoplegia,  Argyll-Robertson  pupil)  is  a  frequent  sign  of  syphilis.  Its 
mechanism  has  been  discussed.  It  is  often  unilateral  in  the  beginning 
of  a  tabes  or  paresis,  or  other  type  of  cerebrospinal  syphilis  and  may 
occur  in  a  number  of  other  conditions,  though  rarely.  In  cerebral 
syphilis  it  usually  becomes  double. 

Isolated  ptosis  may  also  be  seen.  This  may  be  due  to  oculo- 
motor invasion  (tabes),  or  it  may  be  hysterical.  In  the  former  case 
there  is  usually  a  compensatory  overaction  of  the  corrugators,  in 
the  latter,  this  is  less  likely  to  take  place.     Sympathetic  disease, 


202 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


ptosis  from  palsy  of  Miiller's  muscle,  is  usually  accompanied  by 
pupillary  disturbances  and  the  eyeball  lies  somewhat  retracted  in  the 
eyeball  cavity.     The  cervical  sympathetic  is  here  implicated. 

Chronic  Progressive  Eye  Palsies. — These  make  up  a  special  group, 
occasionally  congenital,  more  often  they  are  a  part  of  a  progressive 
anterior  poliomyelitis.     (Bulbar  palsy,  q.  v.) 

Fourth  Nerve  Palsy. — The  fourth  (trochlearis)  nerve  supplies  the 
superior  oblique  muscle  with  its  motor  fibers.  Afferent  fibers  carry 
deep  sensibility  fibers  from  the  muscle.  The  fibers  are  crossed 
and  uncrossed,  the  latter  being  phylogenetically  the  first  to  appear, 
but  later  are  overshadowed  by  the  crossed  fibers.  Isolated  palsy 
causes  a  marked  diplopia,  and  some  dizziness  when  the  patient  looks 
downward  and  outward.  The  false  image  stands  lower  and  nearer 
than  the  true  one,  its  upper  end   inclined   toward  the  true  image. 


Fig.  92. — Paralysis  of  the  fourth  nerve. 
InabUity  to  look  down.  It  will  be  noted 
that  as  the  eyeball  does  not  turn  downward 
the  eyelid  does  not  descend,  at  the  same 
time  the  patient  can  close  his  eyes  when 
told  to  do  so.     (Russel). 


Fig.  93. — Paralysis  of  the  fourth  nerve. 
The  same  patient  closing  his  eyes  to 
command.      (Russell.) 


Looking  upward  or  downward  causes  no  diplopia.  These  patients 
have  difficulty  in  descending  stairs,  and  they  incline  the  head  forward 
and  toward  the  sound  side  to  adjust  to  their  diplopia. 

The  fourth  nerve  is  frequently  involved  with  the  third  and  sixth 
in  basal  inflammations  or  new  growths,  or  may  be  involved  inde- 
pendently from  pressure  in  the  posterior  fossa  (cerebellar  tumor).  It 
also  is  involved  at  its  nuclear  origin  from  encephalitis,  poliomyelitis, 
toxemias,  etc.     (Figs.  92  and  93.) 

Sixth  Nerve  Palsy. — This  is  perhaps  the  most  frequent  of  the  eye 
palsies.  The  peripheral  motor  neuron  is  exposed  for  three-fourths  of 
an  inch  or  more  on  the  base  of  the  skull,  and  is  therefore  subjected 
to  greater  possibility  of  local  pressure  than  any  other  cranial  nerve. 
When  the  external  rectus  muscle  is  paralyzed  there  is  an  internal 
strabismus  and  a  slightly  smaller  pupil  in  the  affected  eye.  There  is 
also  a  diplopia. 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


203 


Sixth  nerve  palsy  more  usually  results  from  basilar  disease,  fracture 
of  base,  meningitis  (syphilitic  and  other  types) ,  tumors  not  only  of  the 
base  but  also  of  the  brain  substance  itself.  Nuclear  involvement  is 
seen  in  encephalitis,  poliomyelitis,  toxemias,  etc.  External  rectus 
palsy  as  a  result  of  a  myositis  is  not  unknown. 

Central  Motor  Neurones. — Isolated  eye  palsies  are  due  to  nuclear 
or  to  peripheral  involvement  of  the  third,  fourth  and  sixth  nerves. 
Supranuclear  disease,  involving  the  oculomotor  paths,  does  not  result 
in  the  loss  of  function  of  a  single  eye,  much  less  of  a  single  eye  muscle. 
Such  lesions  between  the  oculomotor  cortex  and  the  nuclei  in  the 
midbrain  cause  complicated  disorders  of  the  associated  movements  of 
the  eyes.  The  most  frequent  of  these  are:  (a)  conjugate  deviation, 
(6)  lateral  associated  palsy,  (c)  vertical  associated  palsy,  (d)  loss  of  con- 
vergence, (e)  central  nystagmus,  (/)  irregular  types.   (See  Plate  VIII.) 


Fig.  94. — Paralysis  of  upward  movement  of  the  eyes,  showing  the  excessive  wrinkling 
of  the  forehead  in  the  attempt  to  look  up.     Skew  deviation.     (Holmes.) 


(a)  Conjugate  Deviation. — Here  both  of  the  eyes  are  directed  to 
the  side  of  the  lesion,  and  cannot  be  voluntarily  moved  in  an  opposite 
direction.  Yet,  if  the  eyes  are  fixed  upon  an  object  and  the  head 
is  turned  away  from  the  lesion,  the  eyes  will  turn  in  the  direction 
which  voluntarily  is  impossible.  This  is  termed  conjugate  deviation 
of  the  eyes  and  head.  The  eye  axes  may  not  be  truly  parallel, 
but  may  diverge  slightly.  In  acute  apoplexies  this  symptom  is 
occasionally  seen, — i.  e.,  forced  deviation  of  the  head  to  the  side  of 
the  lesion. 

Lesions  of  the  inferior  parietal,  angular  gyrus,  and  possibly  the  foot 


EXPLANATION  OF  PLATE  VIII. 

The  Oculorotary  Paths  and  in  Particular  the  Innervation  of  the 
Muscles  which  Turn  the  Head. 

Ahbreviaiions.—BrQa,  arms  of  anterior  coi'pora  quadrigemina;  C,  the  cochlea  with 
spiral  ganglion,  the  cochlear  branch  of  the  VIII  pair;  Ca,  anterior  horn  of  the  spinal 
cord;  Cge,  external  geniculate;  Cj,  juxta  restiform  body;  Cio{Rm)  interolivary  region 
of  the  medulla  containing  the  median  lemniscus;  Cirl,  retrolenticular  segment  of  the 
internal  capsule;  CoN,  corticonuclear  contingent  of  the  pyramidal  tract,  CSgt,  sag'ttal 
view  of  the  occipitotemporal  lobes;  CV,  visual  area  of  the  internal  face  of  the  hem- 
isphere, transparent  view;  CI-II,  first  and  second  cervical  pair;  DC,  center  of  conjugate 
deviation  of  the  head  and  of  the  eyes;  Dext,  right  external  rectus  muscle;  Dint,  right 
internal  rectus  muscle;  Fa,  ascending  frontal  convolution;  Fi,  F2,  Fz,  three  frontal 
convolutions;  fap,  posterior  and  internal  arcuate  fibers  of  the  medulla;  Flp,  posterior 
longitudinal  fasciculus;  fts,  tectospinal  fibers;  xVi,  central  or  secondary  trigeminal 
pathway;  GG,  Gasserian  ganglion;  G.Sc,  Scarpas  ganglion;  NBe,  Bechterew's  nucleus; 
ND,  Deiters'  nucleus;  NVs,  sensory  trigeminus  nucleus ;  NIII,  oculomotor  nucleus;  III 
pair;  NVI,  nucleus  external  rectus;  NVIIIc,  anterior  terminal  nucleus  of  the  cochlear  ; 
NVIIIv,  triangular  nucleus  of  the  vestibular;  NX  I,  spinal  accessory  nucleus  (trapezius- 
sternocleidomastoid) ;  Os,  superior  olive;  Pi,  P2,  superior  and  inferior  parietal  lobe; 
Pa,  ascending  parietal  convolution;  Pc,  angular  gyrus;  Pci,  inferior  cerebellar  peduncle; 
Pcm,  middle  cerebellar  peduncle;  Pul,  pulvinar;  Qa,  Qp,  anterior  and  posterior  corpora 
quadrigemina;  R,  fissure  of  Rolando;  RgRm,  region  of  median  lemniscus;  Rl,  lateral 
lemniscus;  Rm,  median  lemniscus;  SgR,  gelatinous  substance  of  Rolando;  SR,  reticular 
substance;  SRg,  gray  reticular  substance;  Ti,  Tt,  Tz,  temporal  convolutions;  Tr,  trapezoid 
body;  Y,  vestibular  nerve;  F1V2V3,  three  branches  of  the  trigeminus,  ophthalmic, 
superior  and  inferior  maxillary;  Ysd,  descending  root  of  the  trigeminus;  xM,  tegmental 
crossing  of  Meynert;  XII,  optic  chiasm;  III,  oculomotor;  YI,  external  rectus;  YIIIc, 
cochlear  branches  of  the  auditory;  YIIIv,  vestibular;  XI,  spinal  accessory. 

The  tegmentum  in  its  medullary,  pontine,  peduncular  portions  seen  in  projection  at 
the  level  of  the  aqueduct  of  Sylvius  and  the  fourth  ventricle,  with  the  reticular  formation 
(SR),  the  posterior  longitudinal  fasciculus  (Flp)  and  the  median  lemniscus  {Ryn).  It 
is  limited  laterally  by  the  lateral  lemniscus  {Rl),  colored  in  yellow,  and  the  long  sensory 
nuclei  of  the  trigeminus  (F)  and  of  the  auditory  (YIII)  nerves  {NYs,SgR)  colored  green, 
and  in  yellow  {NYIIIc,  NBe,  NYIIIv,  ND)  and  showing  each  side  of  the  median  line: 
(1)  above  the  nuclei  of  the  ///  pair  (NIII)  which  innervates  by  crossed  and  by  direct 
fibers  the  internal  rectus  of  the  eye;  in  the  center,  the  nuclei  of  the  YI  pair  (NYI)  which 
innervates  the  external  rectus  of  the  eye,  and  (3)  below,  the  cephalorotary  nuclei  which 
act  to  rotate  and  incline  the  head  and  neck;  spinal  nuclei  and  spinal  accessory  NX  I, 
and  motor  centers  of  the  cervical  cord  (Ca).  ~v^^ 

Myelinated  early  are  the  fibers  which  unite  the  nuclei  of  the  sixth  and  of  the  third 
pair  and  of  their  associated  fibers  to  enable  the  lateral  movements  of  the  eye  to  take  place 
in  the  early  stages  of  life.  These  internuclear  fibers,  colored  in  red,  take  their  origin 
from  small  cells  in  the  nuclei  in  the  oculomotor.  III  and  abducens  YI,  and  pass  by  means 
of  the  posterior  longitudinal  fasciculus;  the  small  ganglion  cells  of  the  nucleus  of  the 
left  YI  pair  for  example,  can  put  into  action  the  crossed  and  direct  root  fibers  going  to 
the  left  internal  rectus;  and  at  the  same  time  the  ganglion  cells  of  the  nucleus  of  the 
III  left  pair,  can  put  into  action  the  root  fibers  of  the  homolateral  external  rectus  of 
the  same  side  (left).  Thus  there  is  established  a  strict  physiological  association,  per- 
mitting the  action  of  a  dextrorotary  system,  turning  the  eyes  toward  the  right,  or  a 
levorotary  system,  turning  the  eyes  to  the  left,  an  association  which  can  be  incited 
and  activated  may  be  by  the  cortex,  or  by  various  sensory  or  sensorial  paths,  labyrinthine, 
tactile  or  optic. 

1.  The  cortical  oculorotary  pathway  (colored  dark  red  in  the  right,  pale  red  on 
the  left)  belongs  to  the  corticonuclear  path  (CoN)  (see  Plats  I,  0)  and  takes  its 
(204) 


PLATE   VlIT 


Pulvinar 


Restiform  body 

Juxta-restiform 

body        Vsd 

XI 


origin  from  the  center  of  deviate  conjugation  of  the  head  and  eyes  {Dc)  situated  at  the 
juxtaposition  of  the  second  and  ascending  frontal  regions.  It  passes  by  way  of  the 
knee  of  the  internal  capsule,  internal  portion  of  the  cerebral  peduncles ;  descends  with  the 
aberrant  fibers  of  the  pyramidal  tracts  (see  Plate  I,  D)  in  the  median  lemniscus  and  passes 
to  the  nuclei  of  the  III  pair,  to  the  nuclei  of  the  VI  pair  of  the  opposite  sides,  and  to  the 
cephalorotary  nuclei  of  the  two  sides. 

2.  The  labyrinthine  oculorotary  pathway,  colored  yellow,  contains  a  vestibular 
oculorotary  path,  for  static  equilibrium,  and  a  cochlear,  or  acoustic,  oculorotary  pathway. 
The  vestibular  oculorotary  pathway  takes  its  origin  in  the  cells  of  the  terminal  nuclei  of 
the  vestibular  root  {NBe,  NVIIIv,ND).  Its  fibers  belong  to  the  posterior  and  interior 
arcuate  fibers  of  the  meduUopontine  tegmentum  (Jap).  They  actionate  the  two  VI 
nuclei  and  give  to  the  posterior  longitudinal  fasciculus  (Flp)  direct  and  crossed,  ascending 
and  descending  fibers.  The  ascending  crossed  and  the  descending  direct  fibers  are  the 
most  numerous.  The  ascending  fibers  actionate  the  nuclei  of  both  III  pairs ;  the  descend- 
ing fibers  actionate  the  head  turning  nuclei. 

The  acoustic  rotary  pathway  is  represented  by  the  cells  of  the  anterior  nucleus 
of  the  acoustic  (NVIIIc)  the  trapezoid  body  (Tr)  the  fibers  destined  to  the  superior 
olive  of  the  same  side  (Os)  and  of  the  crossed  side  and  the  fibers  which  go  in  the' lateral 
lemniscus  (Rl).  This  pathway  can  incite  the  oculorotary  system  by  the  fibers  of  the 
hilum  of  the  superior  olive  which  actionate  (a)  the  motor  cells  of  the  external  rectus 
(VI),  (b)  the  motor  cells  of  the  oculomotor  (III)  acting  on  the  internal  rectus  of  the 
opposite  side  by  the  intermediation  of  small  cells  of  the  intranuclear  oculorotary  system. 
The  labyrinthine  oculorotary  pathways  can  be  activated  not  only  by  peripheral  sensa- 
tions— equilibrium  (vestibular),  auditory  (cochlear),  but  also  by  the  cerebellum,  by 
reason  of  the  system  of  internal  fibers  of  the  semicircular  canals  and  cerebellovestibular 
fibers  which  terminate  in  Bechterew's  and  Deiters'  nuclei.  These  connections  are 
not  figured  in  the  plate. 

3.  Lateral  movements  of  the  eyeballs  can  be  induced  by  sensory  stimuli  acting  on 
the  skin  of  the  head  and  body.  The  central  trigeminal  oculorotary  pathway  (green) 
takes  its  origin  in  the  nuclei  of  the  V  pair  (NVs),  passes  by  the  arcuate  fibers  of 
the  meduUopontine  tegmentum  and  the  posterior  longitudinal  fasciculus,  and  then  is 
divided  into  ascending  and  descending  fibers  which  actionate  the  oculomotor  nuclei 
(III  and  VI)  and  the  cephalorotary  nuclei  (XI,  ant-horn  cells  Co) .  The  central  sensory 
pathway  (black)  can  actionate  the  oculorotary  and  cephalorotary  nuclei  by  fibers  which 
originating  in  the  reticular  formation  (cSR)  ascend  and  descend  in  the  posterior  longi- 
tudinal fasciculus. 

4.  The  visual  oculorotary  pathway,  tecto-spinal,  (fts)  (light  blue)  leaves  the  anterior 
corpora  quadrigemina,  crosses  the  middle  line  at  the  level  of  the  dorsal  decussation  of 
Meynert  (xM)  descends  in  the  prelongitudinal  fascicle,  giving  off  terminal  and  collateral 
fibers  to  the  nuclei  of  the  III  pair  of  the  homolateral  side,  to  the  nuclei  of  the  VI  pair 
and  to  the  cephalorotary  nuclei  (NXI,Ca)  of  the  opposite  side. 

It  is  actionated  in  part  by  the  peripheral  visual  pathway  (retina,  nerve,  chiasm) 
(xIIJI)  (dark  blue)  and  in  part  by  the  central  visual  pathway  in  the  calcarine  fissure 
(visual  center)  and  the  anterior  corpora  quadrigemina.  It  can  also  be  indirectly  stimu- 
lated by  the  efferent  visual  pathway,  which  originating  in  the  primary  optic  centers 
(Pul,Cge)  irradiates  in  the  calcarine  cortex  passing  by  way  of  the  retrolenticular  segment 
of  the  internal  capsule  (Cirl)  and  the  optic  radiation  of  the  occipitotemporal  lobes. 

A  destruction  of  the  cortical  oculorotary  pathway  (right)  for  example,  determines  a 
paralysis  of  the  levo-oculorotary  and  levocephalorotary  systems;  that  is  to  say,  a 
conjugate  deviation  of  the  head  and  of  the  eyes  of  the  opposite  side.  A  lesion  of  the 
labyrinthine  oculorotary  pathway,  left,  will  also  determine  a  paralysis  of  the  levo- 
oculorotary  system  by  abolition  of  the  lateral  movements  of  looking  of  the  direct, 
homolateral  side.  In  both  instances  the  patient  looks  to  the  right  because  of  the 
predominance  of  the  antagonists  (Dejerine). 

(205) 


206 


SENSORI-MOTOR  NEUROLOGY—CRANIAL  NERVES 


of  the  second  frontal  gyrus  may  produce  or  occasion  this  type  of 
forced  position  of  the  eyeball. 

Lesion  of  the  centrum  ovale,  and  of  the  internal  capsule  involving 
the  projection  fibers  of  the  oculomotor  may  cause  conjugate  deviations, 
here  associated  with  hemiplegia  as  a  rule. 

The  chief  lesions  causing  conjugate  deviation  are:  hemorrhage  or 
softening,  abscess,  encephalitis,  occasionally  tumor. 

(6)  Lateral  Associated  Palsy. — -Here  the  eyes  are  unable  to  pass  the 
middle  line.  The  altered  position  of  the  head  as  seen  in  conjugate 
deviation  is  absent  and  movements  of  the  head  are  unavailing  in 
bringing  the  eyes  past  the  middle  line.  Convergence,  however,  may 
remain  intact.    Certain  incomplete  conjugate  deviations  are  found  here. 


Fig.   95. — Cerebral  syphilis.     Exter- 
nal rectus  palsy,  right  eye. 


Fig.   96. — Trochlearis   palsy  in  cerebrospinal 
syphiUs. 


Lateral  conjugate  palsy  is  usually  due  to  a  pontine  lesion  on  the 
side  of  the  palsy,  and  which  implicates  the  abducens  fibers  near  the 
nucleus,  and  the  synapses  of  the  posterior  longitudinal  bundle,  possibly 
Lewandowsky's  tractus  pontis  ascendens.  Pressure  at  a  distance  may 
also  occasionally  cause  a  lateral  conjugate  palsy.   • 

Fractures,  pontine  tumors,  multiple  sclerosis,  softening  (after 
labyrinthine  infection)  are  among  the  causes  of  this  comparatively 
rare  condition. 

(c)  Vertical  Associated  Palsy. — Here  the  motion  of  both  eyes  is 
hindered  only  on  looking  up  or  down — all  other  associated  movements 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


207 


are  possible.  When  there  is  loss  of  ability  to  look  down  usually  the 
eyelids  do  not  descend  as  they  normally  do.  In  some  patients  the 
palsy  is  not  symmetrical,  one  eye  moving  up  or  down  more  than 
the  other. 

Certain  forced  positions  of  the  eye,  one  being  higher  than  the  other — • 
(Magendie-Hertwig  position)  may  be  mentioned  here.  These  are 
present  as  a  cerebellar  sign  (middle  cerebellar  peduncle).  The  side 
involved  is  indicated  usually  by  the  lower  lying  eye.  There  is  also 
nystagmus. 

The  cause  for  the  failure  of  the  b'd  to  respond,  analogous  to 
von  Graefe's  symptom  in  exophthalmic  goitre,  is  not  thoroughly 
understood. 


Fig.  97. — Inequality  of  pupils.  Left 
pupil  larger  than  right.  Cerebral  tumor. 
Immobile. 


Fig.  98. — Tabes.       External  rectus  palsy. 


In  the  majorit}^  of  the  cases  lesions  have  been  found  implicating  the 
corpora  quadrigemina  (Pineal  case)  either  directly  or  by  tumor,  or 
by  direct  pressure.  So-called  hysterical  cases  are  usually  mistakes 
in  diagnosis.  One  such  of  Lewandowsky's  proved  to  be  a  cysticercus 
of  the  corpora  quadrigemina.  A  personal  case  developed  a  sarcoma 
of  the  third  ventricle,  pressing  upon  the  anterior  corpora  quadrigemina. 

(d)  Paralysis  of  Convergence. — As  an  isolated  symptom  this  is  rare, 
it  is  usually  accompanied  by  other  associated  palsies.  It  is  found 
most  frequently  in  multiple  sclerosis.  A  closely  related  phenomenon — • 
weakness  of  the  internus  muscles,  Mobius'  symptom  in  exophthalmic 
goitre,  is  thus  far  difficult  of  explanation. 


208 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


Anatomical  Site 


In  levator  palpebrse. 


In  intraorbital,  intracranial, 
extradural,  intradural  or 
infranuclear  portion  of 
peripheral  nerves 


Known  Cause, 

i  Mechanical    lesions     from 

traumata,  tumor,  basilar 

I      meningitis 

I  Rheumatic  toxic  (?) ;     per- 

[     ipheral  (?) 

fMechanical  lesions  from 
I  traumata;  basilar  men- 
I      ingitis 


Rheumatic     postinfectious 
lesions  (peripheral  ?) .  .  . 


Clinical  Picttjee 
1.  Hypokinesias 


Isolated  Ptosis 


Paresis  or  Paralysis 
one  or  more 
ocular  muscles 


Nuclear. 


Congenital  defect 


Bulbar  palsy 
Syringomyelia 


Mechanical  from   trauma, 
hemorrhage,  tumor 

Defective    nutrition     (em- 
bolism, arteritis) '2.  Coo/'dlnatibiJ/p.isturbances 


Convergence  Wealiiiess 
and  Paralysis 


Internuclear  in   Projection! 

I  Stimuli  from  cerebellum 

[stimuli  from  labyrinth. 

[Hemorrhage,     tumor,     en 

Cortical -j      cephalitis,  abscess,  men- 

[     ingitis 


Sympathetic Chiefly  traumatism 

fHysteria, 


Functional. 


Toxic 

Diphtheria,  typhoid .  .  .  ] 
Influenza,  syphilis 
Botulismus 


Tabes,  paresis 
Multiple  sclerosis 


Polioenceph.  Superior .  . 

Lead 

Alcohol 

Cocaine      and      related 
products 

(Hemorrhage,  tumor 


Unilateral  conjugate 
palsy 


Total  Asynergia 
of  eyeballs 


[Neurasthenia,  Basedow.  .. 
Fig.  99. — Summary  of  disturbances  of  external  ocular  nerves.     (Veraguth.) 


DISEASES  OF   THE   TRIGEMINAL  NERVE  209 

(e)  Central  Nystagmus. — The  extremely  complicated  subject  of 
central  nystagmus  is  more  fully  discussed  in  the  section  on  vestibular 
disease.  When  rhythmic,  i.  e. ,  possessing  a  quick  and  a  slow  excursion, 
it  is  usually  vestibular.  Undulating  nystagmus,  i.  e.,  with  uniform 
backward  and  forward  movements,  is  more  apt  to  be  due  to  involve- 
ment of  the  central  or  peripheral  eye  muscle  nervous  pathways.  Pos- 
sibly vestibular  associations  must  always  be  involved.  Undulating 
nystagmus  is  occasionally  seen  in  severe  fatigue,  in  myasthenia,  in 
progressive  muscular  atrophy,  alcoholism,  hydrocephalus,  etc.  Undu- 
lating nystagmus  as  well  as  dissociated  eye  movements  is  normal  in 
infants,  and  is  frequently  seen  in  congenital  defects — (idiots,  imbeciles, 
congenital  blindness) . 

(/)  Irregular  Palsies. — Dissociated  eye  movements  in  which  the 
eyes  move  irregularly,  each  according  to  its  wish,  as  in  Crustacea, 
in  the  very  young  infant,  in  congenital  defective  developments,  is 
seen  coming  on  in  adults  from  destruction  of  the  associative  mechan- 
ism of  the  eye  movements,  more  particularly  from  separation  of  the 
nuclei  of  the  oculomotorius.  Lesions  which  cut  the  nuclei  apart 
one  from  another  (multiple  sclerosis,  tumor)  will  cause  this  asynergia 
or  ataxia  of  the  eye. 

Cycloplegia  is  a  condition  of  absolute  paralysis  of  both  eyes,  the 
gaze  being  fixed  and  immobile,  and  there  being  no  movement  of  the 
iris  either.  A  double-sided  lesion  of  the  corticopontine  eye  muscle 
projection  fibers  causes  such  a  lesion. 

Skew  deviations  are  conditions  in  which  one  eye  is  directed  out- 
ward and  downward,  the  other  inward  and  upward.  Such  a  compul- 
sory eye  position  is  usually  either  due  to  a  middle  cerebellar  peduncle 
affection  or  to  a  cerebellar  lesion  elsewhere. 

Eyeball  apraxias,  so-called,  or  ideomotor  dissociated  movements 
offer  certain  complex  analogies  with  similar  disturbances  of  the  tongue 
muscles  in  speech,  the  facial  muscles  in  mimicry,  or  the  arm  muscles 
in  expression.  They  are  usuall}^  due  to  lesion  of  the  projection  fibers 
in  the  centrum  ovale  or  internal  capsule. 

DISEASES  OF  THE  TRIGEMINAL  NERVE. 

Fifth  or  Trigeminal  Nerve. — ^The  symptomatology  of  lesions  of  the 
fifth  nerve  is  diverse,  as  it  has  both  a  sensory  and  a  motor  part,  and  has 
many  synaptic  junctions  with  cranial,  spinal  and  sympathetic  nerves.^ 

Motor  Part. — The  cortical  origin  of  the  motor  part  is  bilateral,  and 
is  located  in  the  lower  third  of  the  central  convolution.  From  here 
the  fibers  pass  through  the  corona  radiata,  enter  the  internal  capsule 
with  the  pyramidal  tract,  and  make  their  first  synapsis  with  the  chief 
motor  nuclei,  in  the  dorsolateral  part  of  the  tegumentum  of  the  pons. 
Most  of  the  fibers  cross  about  the  level  of  the  posterior  corpora 
quadrigemina.     From  here  the  second  motor  neurone  passes  with  the 

1  Map  scheme  of  the  Sensory  Distribution  of  the  V.  Nerve.     L.  H.  Pegler,  1914. 
14 


210  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

inferior  maxillary  branch  through  the  foramen  ovale,  and  is  dis- 
tributed to  the  masseter,  temporal,  pterygoids,  tensor  tympani, 
tensor  veli  palatini,  mylohyoid  and  the  anterior  belly  of  the  digastric. 

Affection  of  the  cortical  motor  neurons  occurs  in  pseudobulbar 
palsy.  Here  the  lesion  is  bilateral  also.  Unilateral  interruption  of 
the  tract  causes  little  disorder  in  mastication.  (Hirt  claims  that  a 
left-sided  lesion  may  cause  bilateral  palsy.)  Bilateral  disorder  is 
nearly  always  associated  with  the  other  features  of  pseudobulbar 
palsy  {q.  v.).  The  paralytic  signs  are  the  half-open  mouth,  with 
inability  to  close  the  teeth.  The  jaw  cannot  be  protruded,  and  the 
lateral  movements  are  impaired.  The  food  is  apt  to  fall  out  of  the 
mouth,  cannot  be  held  by  the  lips,  and  cheeks  or  tongue,  and  has  to 
be  manipulated  by  the  fingers.  Food  often  is  pushed  up  to  the  pharynx 
and  nose.  There  is  no  atrophy  of  the  muscles  of  the  jaw,  and  no 
reaction  of  degeneration.     The  jaw  jerk  is  increased. 

Cortical  foci  may  give  rise  to  chattering  movements  of  the  jaw. 
Grinding  movements  of  the  jaw,  so  frequent  in  paresis  and  occasionally 
present  in  senility,  are  due  to  cortical  irritation.  The  champing 
movements  of  the  jaw  in  paralysis  agitans  are  possibly  to  be  interpreted 
similarly  to  the  general  tremor  of  the  other  muscles;  namely,  as  an 
interruption  of  the  tonic  impulses  passing  through  the  midbrain 
structures.  (See  Paralysis  Agitans.)  Grinding  of  the  jaw  is  not 
infrequent  as  a  reflex  in  children,  and  it  occasionally  is  seen  as  a  result 
of  basal  meningeal  irritation  of  the  motor  root,  as  in  tuberculosis, 
syphilis,  or  even  tumor  formation. 

Prolonged  spasm  of  the  muscles  of  mastication  is  seen  in  certain 
toxemias,  such  as  strychnine  poisoning,  tetanus,  tetany.  Here  the 
interpretation  is  not  simple.  It  is  a  result  possibly  of  the  marked 
lowering  of  the  synaptic  threshold  in  the  pontine  motor  nuclei,  causing 
over  response  to  the  cerebral  or  reflex  motor  impulses.  The  violent 
convulsive  movements  of  the  epileptic  discharge  are  cortical  in  origin. 

Irregular  or  anomalous  spasmodic  movements  of  the  jaws  occur 
in  multiple  sclerosis,  usually  from  midbrain  or  pontine  localizations 
of  the  plaques,  or  they  may  be  reflex  or  psychogenic  (hysteria  or 
dementia  precox).  In  the  latter  instances  the  biting  symbolizes,  in 
a  few  cases  analyzed,  hate  or  sadistic  complexes.  The  negativistic 
clenching  of  the  jaw  in  food  refusal  as  in  dementia  precox,  depressed 
manic-depressives,  fever  deliria  or  confusion,  expresses  various  sym- 
bolizations.     Fear  of  being  poisoned  is  here  a  frequent  motive. 

Nuclear  disease  of  the  motor  neuron  of  the  trigeminus  may  be 
unilateral  or  bilateral,  partial  or  complete.  In  unilateral  monoplegia 
masticatoria,  the  lateral  movement  of  the  jaw  takes  place  to  the 
paralyzed  side.  Bilateral  lesion  causes  the  jaw  to  fall,  and  abolishes 
all  lateral  movements.  The  floor  of  the  mouth  is  flaccid  from  the 
mylohyoid  and  digastric  palsy,  and  there  is  difiiculty  of  hearing  notes 
of  low-pitched  tuning  forks.  The  muscles  show  atrophy,  reaction  of 
degeneration  and  the  jaw-jerk  is  absent. 


DISEASES  OF   THE   TRIGEMINUS 


211 


Peripheral  trigeminus  motor  palsy  is  usually  associated  with  sensory, 
sympathetic  and  taste  phenomena. 

Nuclear  disease  of  the  motor  neurons  is  comparatively  rare.  It 
may  occur  in  multiple  sclerosis,  in  syphilis  of  the  pons,  hemorrhage, 


i  Anterior  corpora 
Quadrigemina  \  posterior  corpora 


Fig.  100. — Scheme  of  intracerebral  trigeminus  pathways.  Cs,  left  hemisphere;  Cd, 
right  hemisphere;  Th-cort.,  thalamocortical  trigeminus  tracts;  Th,  synapses  of  trigem- 
inus in  thalamus;  Sch,  mesencephalic  leinniscus;  tnot.  Trig.,  motor  trigeminus  root 
nuclei  in  midbrain;  mot.  Tr.  K.,  motor  trigeminus  nucleus  in  locus  ceruleus;  Rad.  mot., 
motor  roots;  Ggl.  Gass.,  Gasserian  ganglion;  I,  II,  III,  three  trigeminus  branches; 
m.s.,  mesencephalic  sensory  trigeminus  roots;  sp.  Tr.  W.,  spinal  trigeminus  branches; 
<S.  gel.  Rol.,  substantia  gelatinosa  Rolando.  Dotted  line,  motor,  solid  line,  sensory. 
(Veraguth,  Bing.) 


poliomyelitis,  syringomyelia.  Peripheral  palsies  are  more  frequent, 
and  are  due  to  trauma,  to  pressure  of  carotid  aneurisms,  tumors, 
chronic  meningitis,  rarely  to  an  interstitial  neuritis. 

Sensory  Part. — Affections  here  are  much  more  intricate  and  complex, 
and  are  often  combined  with  motor  symptoms.     The  sensory  receptors 


212 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


of  the  trigeminus  are  widely  distributed  over  the  face,  the  mucous 
membranes  of  the  superior  and  anterior  nasal  fossa,  the  frontal  and 


A^f///.^^. 


Fig.  101. — Pontine  syndrome.  Alternate  hemianesthesia  of  the  trigeminus  by  lesion 
of  the  median  portion  of  the  left  pontine  tegmentum  involving  the  motor  and  sensory 
roots  of  the  trigeminus,  their  root  fibers,  the  external  portion  of  the  median  lemniscus, 
the  crossed  sensory  paths  of  the  tegmentum  and  extending  as  far  as  the  superior  cere- 
bellar peduncle  behind  and  in  front  as  far  as  the  pons  implicating  peduncular  pyramidal 
fibers.  On  the  right  side  there  is  hemiparesis  of  the  extremities  and  of  the  inferior 
facial  (oblique  cross  hatchings),  hemianesthesia  of  the  extremities,  neck  and  head 
especially  of  pain  and  temperature  sense  (vertical  lines) .  On  the  left  side  there  is  anes- 
thesia of  the  trigeminus;  paralysis  of  the  masticators,  the  pterygoids  especially,  the 
masseter  and  the  temporal.  There  are  choreo-athetoid  movements  of  the  body  due  to 
lesion  of  the  superior  cerebellar  peduncle   (Pes).     (Dejerine.)     See  Midbrain  Section. 


DISEASES  OF  THE  TRIGEMINUS  213 

ethmoid  sinuses,  tentorium  cerebelli,  teeth,  mucosa  of  posterior 
inferior  nares,  the  sinuses  of  the  jaw,  the  dura  mater,  the  mucous 
membranes  of  the  lips,  cheeks,  posterior  and  inferior  portion  of  the 
moutli,  and  anterior  two-thirds  of  the  tongue  as  taste  buds.  The 
sensory  ganghon  is  the  Gasserian. 

Collateral  synapses  occur  with  the  ciliary  ganglion  for  the  passage  of 
impulses  from  the  corneal  and  sclera  receptors.  Impulses  from  the 
ethmoid  and  sphenoid  sinuses;  from  the  pharynx,  posterior  nares, 
hard  and  soft  palates,  maxillary  sinus,  uvula,  pharyngeal  walls, 
tonsils  and  related  mucous  parts  pass  by  way  of  the  sphenopalatine 
ganglion  and  come  into  relation  with  the  glossopharyngeal.  The 
submaxillary  ganglia  connections  are  involved  and  probably  pass 
with  the  vegetative  nervous  system  fibers. 

The  chief  reflexes  arising  from  these  connections  are : 

1.  Winking  reflex. 

2.  Pupillary  (sympathetic  reflex) — pinching  the  cheek  or  neck 
causes  a  dilatation  of  the  pupil  on  the  same  side. 

3.  Jaw  reflex. 

4.  Sneezing  reflex. 

5.  Pharyngeal  reflex — (gagging  and  swallowing) . 

The  centripetal  pathways  from  the  Gasserian  ganglion  join  to 
form  a  large  sensory  root  which  is  distributed  to  two  main  end 
stations:  a  mesencephalic  and  a  spinal  one  with  numerous  collaterals. 
From  these  roots  the  second  sensory  neurone  passes  through  the 
median  lemniscus  to  the  opposite  side  to  end  in  the  thalamus.  A 
further  neurone  then  passes  to  the  sensory  brain  area. 

The  chief  sensory  symptoms  of  fifth  nerve  involvement  are  hyper- 
esthesise  and  neuralgia,  hypesthesise,  anesthesise,  loss  of  taste,  trophic 
and  secretory. 

The  most  frequent  of  the  syndromes  is  trigeminal  neuritis  or  tic 
douloureux  (q.  v.).  Here  the  Gasserian  ganglion  is  often  involved 
or  the  neuralgia  may  be  due  to  pressure  upon  one  or  all  of  the  roots. 
The  distribution  of  the  hyperesthesia  is  of  value  in  determining  the 
branch  or  branches  involved.  It  is  comparatively  rare  to  find  reflex 
neuritic  pains  from  disease  of  the  teeth,  hence  the  hope  that  removal 
of  healthy  teeth  will  cure  a  tic  douloureux  is  usually  doomed  to  dis- 
appointment. This  is  an  extremely  common  error  and  needs  to  be 
emphasized. 

Central  or  thalamic  trigeminal  pains  are  possible.  Clinically 
little  is  known  of  them.  Trigeminal  agnosia  is  a  curiosity  merely. 
Anesthesia  may  be  due  to  interruption  of  peripheral,  pontine,  thalamic 
or  cortical  pathways.  The  diagnosis  as  to  localization  must  be  made 
on  the  basis  of  the  accompanying  symptoms,  sensory,  secretory, 
trophic,  and  motor. 

An  inflammation  of  the  Gasserian  ganglion  causes  a  trigeminal 
herpes  zoster.  Dryness  of  the  eyes,  with  hyperesthesia  or  anesthesia 
is  due  to  a  peripheral  lesion  of  the  superior  or  first  branch  of  the  nerve. 


214         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


sa 


g.se^s 

K&=3     ^ 

iaaa° 

OW.2.S  £ 

W              tH^-t      ^i 

^silf 

oo^cog 

J  M  d  0  m  Ml 

5  c:  S  C'S  c3 

ECHAK 

or     te 
other 
tid, 
throm 

a 

I    a 
•3  iS 


> 


PLATE    IX 


Superposition   of  the    Peripheral    Sensory   Distribution    (in  red) 
and  of  the   Nuclear  (in  black)  of  the  Trigeminus. 

Vl-5,  shows  gradual  involvement  of  the  area  supplied  by  the  trigeminus  in  a  case  of 
syringobulbia;  Vi,  Vii,  Vui,  the  three  branches  of  the  trigeminus;  Ci-v,  distribution  of  the 
cervical  nerves ;  Gg,  the  territory  of  the  geniculate  ganglion  (Hunt's  zoster  zone  of  the 
facial).     (Dejerine.) 


DISEASES  OF  THE  FACIAL  NERVE 


215 


Unilateral  myosis  may  also  point  to  trigeminal  irritation  here.  Related 
dryness  of  the  mucous  membranes  of  the  nose,  lips  and  cheeks,  with 
anesthesise,  usually  point  to  peripheral  disease  of  the  second  branch, 
while  taste  impairment  of  the  anterior  two-thirds  of  the  tongue  may 
be,  but  not  invariably,  associated  with  lesions  of  both  second  and 
third  branches. 

In  root  lesions,  the  epicritic  loss  is  usually  less  than  the  protopathic 
loss,  while  the  reverse  is  usually  true  for  peripheral  lesions.  Pontine 
lesions  show  a  more  general  loss  of  epicritic  sensibility  on  the  side  of  the 
lesion  with  mono-  or  hemi-hypesthesise  or  anesthesise  on  the  opposite 
side  of  the  body,  while  thalamic 
lesions  are  associated  often  with  anes- 
thesia and  analgesia  to  pin  prick, 
central  pain  and  affective  over- 
response. 

Trophic  disturbances,  usually  due  to 
peripheral  disease  (?),  cause  changes 
in  the  gums  and  mucous  membranes, 
ulcerations,  herpetic  eruptions.  Cor- 
neal ulceration  and  loosening  of  the 
teeth  are  often  present,  but  whether 
trophic  or  not  is  not  certain. 

Dissociation  of  pain  and  tempera- 
ture from  epicritic  touch  sensibility 
may  take  place  in  the  trigeminus 
distribution.  For  lack  of  space  here 
a  complete  analysis  of  sensibility  dis- 
turbances of  the  trigeminus  should 
be  sought  in  special  monographs. 
(See  Lewandowsky,  Handbuch  der 
Neurologie,  for  complete  literature — 
1910-1912.) 

Progressive  Facial  Hemiatrophy. — 
This  rare  condition,  which  shows  at 
its   onset    a    gradual  thinning,  with 

wrinkling  of  the  skin  about  the  orbit  or  jaws,  with  later  progressive 
atrophy  of  the  bones,  cartilages  and  muscles,  also  of  the  tongue  and 
soft  palate,  without  sensory  signs  or  reaction  of  degeneration  is  at 
times  a  result  of  peripheral  or  pontine  (nuclear)  disease  of  the  fifth 


Fig.  103. — Syringomyelia,  beginning 
as  hemifacial  atrophy,  then  developing 
"Morvan's  disease,"  and  finally  show- 
ing classical  syringomyelic  end  lesions. 
(Hammond.) 


nerve. 


DISEASES  OF  THE  FACIAL  NERVE. 


Seventh  Nerve. — The  seventh  nerve  is  a  mixed  nerve.  The  cortical 
origin  of  the  motor  neurone  occupies  the  lower  third  of  the  precentral 
convolution,  from  here  the  fibers  pass  through  the  knee  of  the  interna' 
capsule,  through  the  middle  third  of  the  peduncle  and  make  theii 
first  junction  (possibly  by  means  of  intercalated  neurones)  with  the 


216  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

homo-  and  contralateral  seventh  nerve  nuclei  in  the  tegmentum  of 
the  pons,  just  ventrolateral  to  the  abducens  nerve  nucleus.  From 
these  nuclei,  four  in  number  are  usually  described,  the  second  motor 


Fig.  104 — General  scheme  of  the  course  of  the  pyramidal  fibers  and  the  central  paths 
of  the  motor  trigeminus  and  facial.  Vm.,  under  portion  of  the  trigeminus;  VII,  root 
fibers  of  the  facial;  /,  central  facial  fibers;  NV.,  motor  fifth  nucleus;  NVII,  facial 
nucleus;  p,  corticospinal  fibers,  lateral  and  anterior;  ra,  anterior  roots;  t,  central  tract 
of  the  motor  fifth.     (Bechterew.) 


neurone  fibers  make  a  dorsal  upward  curve  (genu  facialis)  around 
the  abducens  nucleus,  then  pass  ventrally  and  emerge  at  the  posterior 
border  of  the  pons,  lateral  to  the  olive,  where  they  lie  in  close  relation 
to  the  fifth  and  eighth  nerves  in  the  cerebellopontine  angles.     They 


DISEASES  OF   THE  FACIAL  NERVE 


217 


are  finally  distributed  (three  ventral  nuclei)  to  the  muscles  of  expression 
of  the  face,  to  the  muscles  of  the  external  ear,  and  of  the  stapedius, 
the  posterior  belly  of  the  digastric  and  the  stylohyoid.  The  frontalis, 
corrugator  supercilii,  and  orbicularis  palpebrarum  are  innervated  by 
fibers  coming  from  the  dorsal  group. 

In  its  peripheral  distribution  the  nerve  passes  through  the  facial 
canal  in  the  temporal  bone  (acqueduct  of  Fallopius),  coming  into 
intimate  relations  with  other  cranial  nerves,  eighth,  pars  intermedia. 


Kueleus  Salioatorlus 


Nucleus  of 
Facial  N. 


^Afferent  divi. 
'  of 

N.Intermediu, 
ending  in 
Glossopharyngeal 
Nucleus 


Superior  Maxillary  N. 
Vidian  N. 
Large  Superficial  Petrosal\    L_^ 

External        '     ' — -^;- --A J^V-  Spheno- 

Superficial  Petrosal ^^^^  \r^ "  palatine 

\>  'i  Ganglion 


To  Auricular' 
Branch  of  t'agus  N. 


Post 
Auricular  Br.^^ 


To  Digastric 
To  Stijlo-hyo 


Efferent  (excito-glandular) 

fibers  to  submaxillary  and 

sublingual  ganglia  and  glands 

Fig.   105. — Plan  of  the  facial  and  intermedius  nerves  and  their  communications  with 

other  nerves.      (Gray.) 

and  also  forming  sympathetic  nerve  associations  of  more  than  usual 
complexity,  a  study  of  which  is  of  value  in  the  local  diagnosis  of  lesions 
of  this  nerve  and  contiguous  parts.     (See  Fig.  104.) 

The  anatomy  of  the  possible  sensory  portion  of  the  nerve  has 
not  been  definitely  homologized.  The  comparative  studies  of  Herrick, 
Johnson  fail  as  yet  to  show  sensory  components  in  forms  higher 
than  the  amphibia.^ 


1  Jour.  Comp.  Neurol,  1914. 


218 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


By  some  its  chief  ganglion  is  considered  to  be  the  geniculate,  which 
is  thought  to  contain  the  efferent  fibers  from  the  receptors  located  in 
the  auricle  of  the  ear,  the  floor  of  the  external  auditory  canal,  the 
t^^mpanum,  and  from  certain  soft  parts  of  the  internal  ear.  The 
nerve  of  Wrisberg  is  considered  to  be  the  sensory  portion  of  the  nerve. 
Hunt's  syndrome  is  a  clinical  picture  thought  to  uphold  this  interpre- 
tation. Again  the  nerve  of  Wrisberg  has  been  described  as  a  mixed 
(thirteenth)  nerve.  From  the  geniculate  two  branches  are  described, 
one  the  chorda  tympani,  and  a  second  entering  the  tract  of  the 
nucleus  solitarius  to  form  part  of  the  gustatory  path. 

Cortical  Palsies. — Cortical  or  capsular  facial  palsy  is  usually  unilat- 
eral, and  most  characteristically  affects  only  the  lower  facial  distribu- 
tion.    The  face  is  drawn  to  the  sound  side,  the  angle  of  the  mouth 

droops  and  the  nasolabial  fold  is 
flattened,  but  the  eyes  can  be  closed 
and  the  forehead  wrinkled.  There 
is  paresis  or  paralysis  of  the  lower 
muscles  varying  with  the  severity 
of  the  lesion.  The  soft  palate  may 
show  palsy,  pulling  to  the  paralyzed 
sideonphonation.  Babinski  further 
described  a  loss  of  the  contractions 
of  the  platysma  of  the  affected  side 
on  forcing  the  mouth  open  against 
slight  resistance. 

In  certain  widespread  cortical 
neurone  palsies,  however,  the  upper 
branches  may  be  involved,  with 
narrowing  (at  times  widening)  of 
the  palpebral  fissure,  and  a  droop- 
ing of  the  outer  angle  of  the  eye- 
brows on  the  affected  side.  Pontine 
syndromes  frequently  show  these 
signs.  See  Midbrain  Section. 
Apraxia  of  the  facial  musculature  is  met  with  in  cortical,  or  corpus 
callosum  lesions.  Here  the  patient  loses  the  power  to  make  proper 
mimetic  movements.  He  may  not  be  able  to  close  the  eye  on  the 
paralyzed  side,  independently  of  the  other.  Furthermore,  in  cortical 
neurone  palsy  the  tongue  protrudes  to  the  paralyzed  side,  or  may  be 
unable  of  protrusion  at  all.     Speech  disturbances  are  frequent. 

In  cortical  facial  monoplegias — or  hemiplegias  with  facial  involve- 
ment— there  are  no  atrophies,  the  electrical  reactions  are  not  involved, 
and  secretory  and  taste  modifications  are  absent. 

Cortical  and  subcortical  irritation  may  give  rise  to  facial  convulsive 
movements— spontaneous  laughing  or  crying  movements. 

The  facial  mimetic  movements  of  purely  psychogenic  origin,  tics, 
etc.,  are  numerous  and  often  closely  resemble  choreic  movements. 


Fig.  106. — Pseudobulbar  palsy. 
(TUney.) 


DISEASES  OF  THE  FACIAL  NERVE  219 

Pontine  Facial  Lesions. — Here  the  nuclei  of  the  peripheral  neurone 
being  involved  one  expects  to  find  all  of  the  branches  affected,  but 
inasmuch  as  there  are  different  groups  of  nuclei,  occasionally,  as  in 
poliomyelitis  for  example,  certain  muscles  are  involved  and  others 
are  not.  A  general  lesion  here  will  cause'  a  total  palsy  of  the  muscles 
with  atrophy  and  loss  of  electrical  reactions.  There  are  no  changes 
in  taste,  nor  hearing  in  the  pure  nuclear  cases. 

Lesions  here  are  apt  also  to  involve  the  third  nerve,  also  the  pyra- 
midal tract  fibers,  and  the  sensory  fibers  of  the  fillet,  hence  a  variety 
of  hemiplegic  or  hemi anesthetic  syndromes — crossed  or  lower  alternate 
hemiplegias.     (See  Section  on  Midbrain.) 

Supranuclear  pontine  lesions  may  rarely  be  double  (Pseudobulbar 
palsy  types). 

Peripheral  Facial  Palsies. — Bell's  Palsy  more  properly  speaking. 
Here  a  variety  of  syndromes  may  occur  depending  on  the  exact  site 
of  the  lesion.  These  may  be  conveniently  divided  into  five  syn- 
dromes: (1)  Most  peripheral,  due  to  disease  or  pressure  at  or  outside 
of  the  stylomastoid  foramen.  This  results  in  a  complete  paralysis  of 
the  muscles  of  the  side  of  the  face.  At  rest  the  asymmetry  is  marked 
in  proportion  to  the  severity  of  the  palsy — all  grades  are  found.  The 
muscles  of  the  forehead  cannot  be  contracted  horizontally  or  vertically, 
the  eye  remains  partly  or  widely  open  on  attempts  at  closure,  closing 
at  night  in  sleep;  the  nasal  orifice  is  narrowed,  the  nasolabial  fold  is 
obliterated,  the  angle  of  the  mouth  droops  and  shows  the  teeth,  and 
there  is  pulling  of  the  mouth  to  the  sound  side.  Puffing  the  cheek  is 
impossible,  holding  food  and  saliva  are  difficult,  and  on  attempting  to 
whistle  the  air  comes  out  on  the  paralyzed  side.  Tears  run  down  the 
cheek  but  the  reddening  of  the  eye  is  secondary.  There  is  less  sweat- 
ing on  the  paralyzed  side.  Pressure-pain  sensibility  is  unimpaired. 
The  palate  and  tongue  may  be  apparently  involved,  but  careful 
scrutiny  shows  otherwise.  Reaction  of  degeneration  sets  in  as  a  rule 
in  the  severe  cases.  Slight  speech  disturbance  is  apparent,  especially 
in  the  beginning,  and  is  very  marked  with  a  (rarely  occurring)  double 
peripheral  palsy  (see  Figs.  107,  108,  109  and  110). 

These  palsies  are  due  to  trauma  or  pressure  from  a  tumor,  possibly 
a  perichondritis  of  or  swelling  about  the  stylomastoid  foramen  (called 
rheumatic  or  refrigeration  palsy).  The  effect  of  cold  upon  the  facial 
nerve  itself,  i.  e.,  by  exposure  in  riding  with  one  side  exposed  to  open 
windows,  etc.,  looms  large  in  statistical  enquiries. 

2.  Just  within  the  stylomastoid  foramen,  and  within  the  Fallopian 
canal,  the  chorda  tympani  travels  with  the  facial  nerve.  A  lesion 
here  will  cause  all  of  the  symptoms  just  enumerated,  and  in  addition 
there  will  be  an  impairment  or  loss  of  taste  of  the  anterior  two-thirds 
of  the  tongue.  There  will  also  be  a  diminution  of  the  salivary  secre- 
tions.    Pressure-pain  sensibility  is  not  impaired. 

3.  If  to  this  last  syndrome  hyperacusis  and  tinnitus  alone  be  added 
the  lesion  is  slightly  further  back  in  the  canal  involving  the  branch 
given  off  to  the  stapedius  muscle  (see  Fig.  104). 


220         SENSORI-MOTOR  NEUROLOGY^CRANIAL  NERVES 

4,  Lesions  lying  between  the  geniculate  and  the  stapedius  within 
or  at  the  internal  entrance  to  the  Fallopian  canal,  cause  a  bewildering 
variety  of  additional  symptoms,  whose  exact  anatomical  relations  are 
still  somewhat  obscure. 


Fig.  107. — Facial  palsy.     Smiling.  Fig.  108. — Facial  palsy.     Whistling. 


Fig.  109. — Facial  palsy.    Closing  Fig.  110. — Facial  palsy.    Showing 

the  eyes.  the  teeth. 

(a)   Geniculate    Syndrome.     Hunt's    Syndrome} — Here    one    meets 
with  a  herpes  of  the  auricle  and  the  external  auditory  canal.     This, 

1  J.  Ramsey  Hunt,    Journ.    of    Nerv.    and    Ment.    Dis.,    1909.      Kidd,    Rev.    Neur. 
Psychiatry,  September,  1914. 


DISEASES  OF   THE  FACIAL  NERVE 


221 


Mi  : 

.S  • 

toxs 

^r;3 

3    . 

o^ 

"  d 

<D 

CO   ^ 

M  «*-< 

J  o 

03 

X! 

1>^ 

sB  ti 

c  rt 

'o 

!^-^         -S-S 


.S  ft 


OT3 

«  5 

O   03 


^  A. 

5H 


gm 


fe  W 


w  H  -< 


t)     O 


222  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

according  to  Hunt,  is  the  zoster  zone  of  the  geniculate.  Extension 
of  the  inflammation,  or  pressure,  causes  a  facial  palsy  plus  the  herpes. 
In  a  more  extensive  process  auditory  symptoms,  tinnitus,  diminution 
or  loss  of  hearing  are  added.  In  rare  instances,  from  involvement 
of  the  vestibularis,  nausea,  vomiting,  nystagmus  and  dizziness  are 
present.  The  chief  causing  lesion  is  an  inflammation  of  the  geniculate 
ganglion.  Occasionally  occipitocollaris  herpes  is  an  associated  phe- 
nomenon. Severe  otalgias  with  or  without  tympanic  herpes  are  also 
at  times  the  expression  of  a  geniculate  involvement. 

Facial  palsy  of  non-geniculate  origin  from  lesions  in  the  same  region 
may  or  may  not  be  accompanied  by  loss  of  hearing.  The  chief  addi- 
tional diagnostic  feature  of  lesions  here  is  the  lowering  of  the  threshold 
of  deep  sensibility  (Maloney). 

Lesions  of  the  seventh  nerve  at  its  emergence  from  the  pons  usually 
implicate  other  structures,  notably  the  fifth  or  eighth  nerves,  at  times 
the  sixth,  eleventh,  twelfth.  The  facial  palsy  is  of  the  peripheral  type 
with  no  loss  of  taste,  changes  in  the  secretions,  or  suppression  of  the 
lachrymal  secretions.  Basal  symptoms  such  as  anorexia,  nausea, 
headache,  disk  changes  are  often  present.  The  chief  pathological 
processes  are  basal  meningitis,  usually  syphilitic,  or  tumor  formation. 

DISEASES  IN  THE  AUDITORY  AND  VESTIBULAR  PATHWAYS. 

The  Eighth  Pair. — The  eighth  cranial  nerve  is  in  reality  two  separate 
nerves,  with  distinctly  different  structures,  pathways  and  functions. 
It  is  not  a  single  nerve  with  two  parts.  The  two  nerves  are  the  coch- 
lear or  auditory  proper,  and  the  vestibular — a  portion  of  the  cere- 
bellar apparatus. 

The  former  handles  sounds,  the  latter  serves  to  orient  the  body  in 
space.  Their  chief  receptors  lie  closely  related  in  the  sphenoid  bone. 
By  reason  of  this  close  topographical  relationship  infections  of  the 
middle  ear  are  apt  to  involve  both  structures,  and  by  reason  of  the  close 
associations  with  intracranial  structures,  brain  involvements,  men- 
ingitis, abscess,  etc.,  may  result.  Their  central  stations  are  wide 
apart  in  the  temporal  cortex  and  cerebellum  respectively. 

Auditory  Nerve. — The  receptors  for  sound  stimuli  lie  in  the  organ 
of  Corti  in  the  cochlea.  They  respond  to  sound  stimuli  of  11  octaves, 
i.  e.,  from  10  to  7840  double  vibrations.  Ordinary  conversational  or 
musical  sounds  usually  lie  within  16  to  4032  vibrations.  Space  orien- 
tation through  sound  is  purely  associative.  The  chief  avenues  for 
sound  conduction  to  the  receptors  is  the  auditory  canal,  but  sound 
conduction  is  also  possible  by  way  of  the  bones,  chiefly  of  the  skull, 
in  part  by  other  bony  structures.  From  the  cochlea  the  branches 
coalesce  to  form  the  acoustic  nerve  which,  passing  in  the  auditory 
canal  with  the  facial,  enters  the  medulla  at  about  the  cerebellopontile 
angle.  The  sensory  ganglion  is  the  tuberculum  acusticum.  The 
further  course  of  the  pathways  is  illustrated  in  Figs.  112,  113,  also 
see  Plate  VIII. 


DISEASES  IN  AUDITORY  AND  VESTIBULAR  PATHWAYS     223 


The  chief  disturbances  of  the  auditory  nerve  are:  (1)  various  forms 
of  deafness.  These  vary  considerably  and  may  be  absolute  or  partial. 
Certain  tones  may  be  cut  out  and  not  others,  upper  or  lower  tones, 


Fig.  113. — Central  acoustic  paths,  na,  anterior  acoustic  nucleus;  ta,  acoustic  tubercle; 
Sta,  acoustic  striae;  os,  superior  olive;  ctr,  trapezoid  body;  nVI,  abducens  nucleus; 
nil,  lateral  lemniscus;  cqi,  cqs,  posterior  and  anterior  corpora  quadrigemina ;  cgi,  exter- 
nal geniculate  body;  st,  acoustic  path  to  cortex;  It,  temporal  lobes;  th,  thalamus. 
(Bechterew.) 


224  S^NSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


sometimes  intermediate  tones  drop  out  (hearing  scotomata,  analogous 
to  optic  scotomata,  are  not  infrequent  in  hysterical  reactions,  dementia 
precox,  in  multiple  sclerosis,  paresis,  tabes,  etc.).  These  anomalies  of 
hearing  are  chiefly  peripheral,  either  in  the  primary  receptors  or 
occasionally  in  the  ganglion.  Paracusia  (buzzing,  whistling,  crackling), 
are  also  peripheral  for  the  most  part,  but  may  also  be  central  as  in 
psychotic  or  psychoneurotic  syndromes. 


Fig.  114. — Scheme  of  the  central  acoustic  pathways.  Ti,  T2,  first  and  second  temporal 
lobes;  J,  island  of  Reil;  ce,  claustrum;  Li,  lenticular  nucleus;  Ci,  internal  capsule; 
Thai.,  thalamus;  U.S.,  median  lemniscus;  S.s.,  optic  radiations;  hint.  V.L.,  posterior 
quadrigemina;  B.a.,  middle  cerebellar  peduncle;  c.c.p.,  crus  cerebelli  ad  pontem; 
c.  gen.  int.,  internal  geniculate;  Cge.,  external  geniculate;  Sta.,  striae  acoustical;  gsp  ., 
ganglion  spirale;    Ta.,  acoustic  tubercle,     (v.  Monakow.) 

Complete  deafness  from  medullary  lesions  is  rare  for  anatomical 
reasons  already  indicated. 

Cortical  or  sub-cortical  involvement  of  the  auditory  pathways  cause 
various  forms  of  amnesic  aphasia.     (See  under  Speech  Mechanism,) 

Chaet  for  Differentiations  of  Peripheral  and  Central  Lesions. 


Disease  of  sound-conducting 

Disease  of  sound-perception 

apparatus. 

apparatus. 

Speech  test. 

Deep  tones  heard  worse  than 

Deep  tones  heard  worse  or 

higher  ones. 

better  than  higher  ones. 

Air  Conduction  for  forks 

Deep  tones  heard  worse  than 

High  tones  heard  worse  than 

C.  cc',   c2,   c\  c* 

and 

higher  ones.       Lower  tone 

deep   ones.       Upper    tone 

for  Galton's  whistle. 

threshold  raised. 

threshold  lowered. 

Weber  (ci). 

Median  or  localized  in  worse 

Median  or  localized  in  better 

ear. 

ear. 

Schwabach  (c^. 

Lengthened. 

Rarely  normal,  mostly  short- 
ened. 

Rinne  (C.  cc',  c^). 

Negative. 

According  to  grade  of  retar- 
dation only  for  C.  or  for  C. 
and  the  higher  tones  to  c^. 

Positive. 

DISEASES  IN  AUDITORY   AND   VESTIBULAR  PATHWAYS     225 

Vestibular. — It  has  been  established,  almost  beyond  question,  that 
the  labyrinth  is  the  chief  organ  of  the  body  connected  with  the 
receiving  of  impressions  of  its  position  in  space,  particularly  for  the 


General  Etiology  of 
Chief  Disturb- 
ances. 

1.  Congenital  defects. 


The  Chief  Primary  Localizations. 


The    Resulting 

Clinical 

Signs. 

jeneral    difficulty    in 
hearing. 


2.  Functional  strain. 


Not  sharply  localized 


3.  Disturbance  of  Psycho- 
tonic  component. 


In  labyrinth  and  in  the 
auditory  Ij-lng  in  the 
sphenoid. 


Partial  deafness  espec- 
ially to  high  notes. 


f  Djafness. 


4.  Mechanical        (trauma, 
hemorrhage;  tumor). 


>  Ringing  in  ears. 


5.  Metabolic  disturbance, 
(a)  Quantitative,    ar- 
teriosclerosis; 
cachexia. 


(6)  Poisoning  from  exo- 
genous sources 
(quinin,  salicylic 
acid,  alcohol, 
CO2,  nicotin, 
lead) . 


In  endocranial  part 
of  the  auditory. 


In  total  peripheral 
nerve. 


Hypcracusis. 


)  Meniere  vertigoes. 


,  Deafness  in  company. 


(c)  Poisoning  from 
parasitic  sources 
(syphilis,  diph- 
theria,  typhoid). ' 


In  central  acoustic 
tract. 


,  Cortical  deafness 
(aphasia). 


(d)  Associated  inflam- 
mations: 
Middle  ear. 
Meningitis. 


In  cortical  auditory  ai-eas. 


6.  Multiple  sclerosis. 

Fig.  115. — Summary  of  auditory  disturbances.      (Veraguth.) 

head.     The  meaning  for  the  human  body  of  the  physical  laws  of 
gravity  is  its  chief  concern.     It  is  adapted  to  the  mechanical  stimuli 
of  gravity,  acting  largely  through  the  otolith  organ,  which  react  to 
15 


226  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

changes  in  the  incidence  and  degree  of  pressure  upon  its  sensory  end 
organs,  due  to  changes  in  the  specific  gravity  of  its  surrounding  fluids; 
and  also  through  the  semicircular  canals  which  react  to  changes  in 
position  in  the  three  planes  of  space.  The  slightest  change  of  the 
body  in  space  is  felt  by  this  apparatus,  and  in  the  healthy  central  nerv- 
ous system  any  such  change  is  automatically  reacted  to  by  appropriate 
motor  response. 

This  motor  response,  however,  is  a  complicated  mechanism,  and  all 
of  its  elements  are  not  thoroughly  analyzed.  One  of  its  parts  is  that 
of  a  reflex  muscular  tonus,  by  which  the  ordinary  posture  of  the  body 
is  maintained.  It  is  this  function  that  has  entitled  it  to  the  appellation 
of  the  labyrinthine  tonus.  Sherrington^  has  analyzed  the  complicated 
interrelations  between  the  proprioceptors  of  the  limbs,  muscles,  joints, 
etc.,  which  carry  impressions  of  movements,  strains,  tensions,  etc., 
and  the  receptors  in  the  labyrinth.  It  is  impossible  to  enter  into  them 
here.  Suffice  it  to  say  that  the  result  is  the  reflex  maintenance  of  the 
posture  of  the  body,  including  the  compensatory  reflexes  of  the  head, 
and  those  muscles  of  the  head  capable  of  changing  the  sense  of 
consciousness  of  position,  particularly  the  muscles  of  the  eyeballs. 

The  labyrinth  belongs  to  a  series  of  organs  that  work  in  re- 
sponse to  gravity.  It  is  a  part  of  a  great  system  of  connections — which 
Sherrington  has  designated  as  the  proprioceptive  system — which  gives 
the  animal,  human  as  well  as  others,  a  definite  attitude  toward  the 
external  world  of  space.  It  is  the  most  important  of  these  organs. 
It  is  connected  in  a  system  with  other  nervous  structures  performing 
their  part  in  the  same  general  function,  and  each  segment  of  the  body 
is  caught  up  in  the  chain  of  connections  from  the  lower  end  of  the  spinal 
cord  to  the  frontal  area  of  the  cortex. 

This  whole  complicated  system  of  end  organs,  fiber  connections, 
long  and  short  fiber  tracts,  has  its  chief  center,  just  as  every  other 
reflex  system  has  its  center.  The  chief  center  or  head  ganglion  of 
this  whole  proprioceptive  system  is  the  cerebellum.  The  cerebellar 
connections  of  the  vestibular  system,  the  vestibulospinal,  vestibulo- 
bulbar,  vestibulocerebellar,  and,  finally,  the  cerebellorubrocortical 
components  which  carry  those  fibers  whose  functioning  is  recognized 
in  the  consciousness  of  space  relations,  are  now  fairly  well  known, 
not  in  their  entirety,  but  in  their  main  tracts  and  connections.  Hence, 
disease  or  disorder  which  shows  any  perturbation  of  the  function  of 
orientation  in  space  may  be  more  or  less  accurately  localized  along  the 
fiber  tracts,  carrying  the  necessary  impulses  underlying  these  functions, 
and  an  appropriate  therapy  adopted  (see  Plates  VIII,  X,  and  XI). 

Nervus  Vestibularis. — The  fibers  of  the  median  acoustic  root  (Lewan- 
dowsky — mixed)  constitute  the  central  prolongation  of  the  bipolar 
ganglion  cells  which  make  up  the  vestibular  or  Scarpa's  ganglion. 
The  peripheral  prolongations  of  the  cells  originate  in  the  walls  of  the 

1  The  Integrative  Action  of  the  Nervous  System. 


DISEASES  IN   AUDITORY  AND   VESTIBULAR  PATHWAYS     227 

semicircular  canals.  The  thick  bundle  of  the  median  root  pushes  its 
way  between  the  spinal  trigeminus  root  and  the  corpus  restiform 
(inferior  cerebellar  peduncle)  lying  at  first  close  to  the  median  edge 
of  the  spinal  accessory  nucleus,  and  reaches  dorsally  like  the  tines  of 
a  fork  toward  the  end  nuclei.  These  end  nuclei  of  the  vestibular  are 
the  triangular  and  the  large  cell  nucleus,  which  latter  consists  of: 
(1)  spinal  acoustic;  (2)  Deiters'  and  (3)  Bechterew's  nucleus.  Cajal 
describes  also  a  crossed  root  of  the  vestibularis  whose  bundles  can  be 
traced  along  the  dorsal  border  of  the  spinal  trigeminus  root  through 
the  raphe  and  can  be  followed  to  the  other  side  (Bechterew). 


\- Nucleus  ruber 


Superior  cerebellar]^     /  /      I 
peduncle 


Dentate 
nucleus 

-Roof  nucleus 


Deiter's  nucleus 


N.  vestibularis 


Tr.  spino-cerebellares 


Tr.  rubro-spinalis 

Tr.  vestibulo-spinalis-- 

Dorsal  longitudinal  \_ 
Bundle  ] 


Fig.   116. — Diagram  to  illustrate  the  spinal  connections  of  the  cerebellum.     On  the 
right  the  afferent  tracts  are  represented,  on  the  left  the  efferent  cerebellar  tracts. 


Of  the  connections  of  the  end  nuclei  of  the  vestibularis  those  of  the 
cerebellum  are  the  plainest.  Strong,  somewhat  swollen  bundles 
of  nerve  fibers  go  from  the  Deiter  and  Bechterew  nuclei  dorsally  in 
the  cerebellum.  Fibers  from  the  nuclei  triangularis  also  join  them. 
The  acoustic  cerebellar  tract  lies  on  the  medial  side  of  the  inferior 
cerebellar  peduncle,  in  the  medial  lateral  portion  from  the  superior 
cerebellar  peduncle,  in  which  a  portion  also  goes.  The  majority  of 
the  bundles  go  to  the  cerebellar  worm  and  end,  mostly  crossed,  in  the 


228 


SENSORI-MOTOR  NEUROLOGY  ^CRANIAL  NERVES 


nuclei  of  the  roof  (tectalis),  probably  also  in  the  nucleus  globosus  and 
nucleus  emboliformis. 

Within  the  superior  cerebellar  peduncle  portions  it  may  be  said 
that,  according  to  Bechterew  and  Flechsig,  the  Bechterew  nuclei 
are  connected  by  means  of  commissural  fibers  which  pass  out  with  the 
superior  cerebellar  peduncle  from  the  cerebellum  and  bend  arcwise 
in  the  posterior  angle  of  the  crossing  of  the  superior  cerebellar 
peduncles. 

Vestibular  Vertigoes. — At  one  time  loosely  grouped  together  under 
the  name  Meniere's  disease,  the  analyses  of  later  years  have  shown 
a  great  variety  of  these  affections  depending  upon  the  anatomical  sites 
of  the  lesions.    One  must  distinguish  between: 

F.  thalatno-corticalis 


Nucleus  ruber 

Superior  cerebellar 

peduncle 


Nucleus 
dentatus 


F.  olivo-cerebellaris^' 


F.  cortico-pontinus 

J  Central  Tegmental 
(  Tract 

Middle  cerebellar  peduncle 

Inferior  Olive 


Fig.  117. — Diagram  to  illustrate  the  afferent  and  efferent  connections  of  the  cerebellum 

with  the  forebrain. 


1.  Disease  of  the  peripheral  end  organ,  (a)  partial,  or  (b)  complete; 
these  are  the  vertigoes  of  partial  or  complete  labyrinthine  disease. 

2.  Disease  of  'the  first  neurone,  (a)  paresis,  (6)  paralysis  of  the 
vestibularis. 

3.  Disease  of  the  primary  end  nuclei  in  the  medulla  and  of  Deiters' 
nucleus.  The  latter  gives  a  special  symptomatology  termed  Bonnier's 
syndrome. 

4.  Disease  in  the  region  of  the  posterior  longitudinal  bundle  asso- 
ciated with  eye  movement  vertigoes. 

5.  Disease  of  the  nuclear  region  of  the  eye  muscles  in  the  corpora 
quadrigemina. 

6.  Disease  of  the  pontine  eye  nuclei. 

7.  Disease  of  central  eye  paths. 

8.  Disease  of  cerebellum. 


DISEASES  IN  AUDITORY  AND  VESTIBULAR  PATHWAYS     229 

In  disease  of  all  these  regions  vertigoes  are  to  be  expected  by  impli- 
cation of  the  vestibular  nerve;  the  character  of  the  accompanying 
phenomena,  especially  the  nystagmus,  determines  the  location. 

In  partial  or  circumscribed  disturbance  of  the  vestibular  end  organs 
in  the  labyrinth  the  vertigo  is  associated  with  nystagmus  movements. 
The  nystagmus  is  spontaneous  and  shows  a  long  slow,  due  to  the 
vestibular,  and  a  quick  return  movement  due  to  the  tegmental  nuclei, 
the  direction  of  the  quick  movement  naming  the  nystagmus.  Vestib- 
ular nystagmus  usually  increases  when  the  eyes  are  directed  in  the 
direction  of  the  quick  movement,  and  usually  diminishes  or  ceases 
on  looking  in  the  opposite  direction.  There  is  almost. always  a  com- 
bination of  horizontal  and  of  rotary  nystagmus.  Barany  states  that 
ever}'  other  form  of  spontaneous  nystagmus  is  of  intracranial  origin. 
If  the  nystagmus  movement  is  rotary  and  horizontal  it  must  be 
determined  whether  it  is  peripheral  or  central.  A  peripheral  nystag- 
mus to  the  right  should  show  on  caloric,  pressure,  and  rotation  tests 
that  the  right  vestibule  is  functionally  active.  Should  such  tests  show 
an  inactive  right  vestibular  then  the  nj'^stagmus  must  be  of  central 
origin.  If  the  right  vestibular  is  active,  then  continued  observation 
of  the  nystagmus  will  alone  determine.  Should  the  nystagmus  con- 
tinue uninterruptedly  for  twenty-four  hours  or  more  it  is  of  intra- 
cranial origin.  If  it  lasts  a  shorter  interval  and  is  uninterrupted  by 
quiet  intervals,  it  may  be  either  peripheral  or  central.  When  there  is 
also  nystagmus  of  the  well  side,  which  lasts  about  two  weeks,  gradually 
decreasing,  then  a  peripheral  disturbance  seems  certain.  Intracranial 
nystagmus  is  not  so  apt  to  diminish. 

The  Meniere-like  attacks  are  either  mild  or  marked.  Buzzing 
in  the  ears  is  rare  in  mild  attacks.  There  is  no  impairment  of  hear- 
ing. In  the  severer  attacks  there  is  little  buzzing,  but  hearing  is  apt 
to  be  impaired.  In  free  intervals  the  nystagmus  diminishes  or  dis- 
appears, the  Barany  caloric  reaction  is  diminished  on  the  affected  side. 

Total  destruction  may  be  acute  or  chronic;  the  latter  may  show 
no  symptoms.  The  former  sets  in  with  violent  vertigo,  nausea,  vomit- 
ing. There  is  marked  horizontal  and  rotary  nystagmus  of  the  sound 
side.  The  slightest  movement  of  the  head  increases  the  vertigo  and 
nystagmus  during  the  first  forty-eight  hours;  the  latter  gradually 
disappears  in  three  to  four  weeks.  There  is  marked  loss  of  coordina- 
tion, with  tendency  to  fall  to  one  or  the  other  side.  After  the  period 
of  quiescence  of  the  nystagmus,  caloric  and  rotation  tests  show  the 
defective  function.    The  galvanic  reaction  is  not  usually  affected. 

Disease  of  the  vestibular  nerve,  usually  due  to  tumor  of  base 
(acoustic,  cerebellopontine  angle),  leads  to  similar  reactions.  Here, 
however,  there  seems  to  be  a  difference  in  that  Neumann  has  found 
that  the  galvanic  reaction  is  reduced  or  lost,  according  to  a  partial 
or  complete  destruction  of  the  vestibular  ganglion.  Other  cranial 
nerves  are  here  involved  as  a  rule.  The  cochlearis  is  frequently  in- 
volved.    Complete  deafness  does  not  result.     The  trigeminus  is  also 


230  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

often  involved  and  pain,  paresthesise,  or  motor  defects  appear.  Cere- 
bellar symptoms  may  also  complicate  the  picture.  The  nystagmus 
is  apt  to  continue  in  intensity  with  tumors,  and  may  be  on  the  sound 
as  well  as  the  affected  side. 

Involvement  of  the  nuclei  (encephalitis,  abscess,  syphilis,  tumor) 
brings  about  similar  attacks  of  nausea,  vomiting,  vertigo,  and  nys- 
tagmus. The  symptoms  continue  and  increase,  as  a  rule,  beyond  the 
three  weeks  ordinarily  seen  in  labyrinthine  disease. 

The  method  of  continuous  observation  aids  in  locating  the  diseased 
focus. 

Bonnier's  Syndrome,  due  to  implication  of  Deiters'  nucleus  and 
contiguous  structures,  usually  causes  a  marked  attack  of  nausea, 
vomiting,  vertigo,  and  nystagmus  with  buzzing  in  ears  and  deafness 
(Meniere's  syndrome),  with  irradiations  to  the  ninth  and  tenth  nerves 
causing  anxiety,  tachycardia,  and  hemiplegic  weakness.  The  trigem- 
inus and  oculomotor  are  also  apt  to  be  involved.  Bonnier  has  also 
described  peculiar  somnolent  attacks  accompanying  his  syndrome. 
Little  can  be  done  for  these  cases  unless  the  focus  is  of  syphilitic  origin. 

Here  vertigo  and  nystagmus  are  associated  in  various  ways,  but  the 
vertigo  disappears  on  closing  the  eyes.  Here  forced  movements 
conjugate  deviations,  and  various  skew  deviations  afford  a  clew  to 
diagnosis.  Caloric  and  other  tests  determine  the  integrity  of  the 
labyrinthine  functions. 

Cerebellar  vertigoes  have  a  number  of  special  features.  So  far 
as  the  vertigo  is  concerned  they  may  not  be  separable  from  the  laby- 
rinthine or  vestibular  vertigoes.  Hearing  symptoms  are  usually  absent. 
The  nystagmus  is  less  apt  to  be  horizontal  and  rotary,  but  may  be  up 
or  down  or  oblique,  and  is  usually  directed  toward  the  affected  side. 

There  are  usually  also  symptoms  of  a  tumbling  gait  toward  the  side 
of  the  lesion;  there  is  asynergia  and  usually  adiadokokinesia.  No 
real  distinction  as  to  the  side  of  the  lesion  affected  can  be  gained  from 
the  fact  as  to  the  subjective  or  objective  motion  of  the  objects  during  a 
vertiginous  attack.  Closure  of  the  eyes  has  no  marked  affect  upon  the 
vertigo,  nor  upon  the  gait.  Caloric  and  other  tests  determine  a  normal 
labyrinth. 

Treatment. — Here  there  comes  into  consideration  the  surgery  of 
the  ear  and  the  surgery  of  the  cerebellum  and  the  cerebellopontine 
angle.  The  ear  specialist  should  treat  the  labyrinthine  cases,  not  the 
neurologist.  Rest  in  bed,  quinin,  and  the  usual  medical  treatment 
which  shuts  one's  eyes  to  the  danger  of  a  suppurative  labyrinthitis, 
brain  abscess,  etc.,  is  folly. 

In  the  apoplectic  form  of  Meniere's  syndrome  (hemorrhagic  laby- 
rinthitis) often  mistaken  for  a  cerebral,  or  cerebellar  hemorrhage, 
the  patient  must  be  kept  absolutely  quiet,  the  eyes  should  be  kept 
closed,  the  room  darkened,  and  all  noises  excluded  as  far  as  possible — 
telephone,  house  bell,  etc.,  shut  off.  Ice  should  be  applied  to  the 
mastoid.     Leeches  are  at  times  of  value.     The  continuous  vomiting 


DISEASES  IN  AUDITORY  AND  VESTIBULAR  PATHWAYS     2.31 


may  be  in  part  relieved  by  swallowing  cracked  ice.     Surgical  inter- 
ference may  be  called  for. 

In  syphilitic  cases  mercurial  injections,  salvarsan,  or  inunctions  are 
called  for.  It  may  be  noted  that  the  acute  labyrinthine  disturbance 
which  has  been  known  to  occur  after  the  use  of  salvarsan  is  probably 
due  to  the  syphilis  and  not  to  the  arsenic  (Benario). 


Fig.  118. — General  scheme  of  the  cerebral  paths  of  the  vestibular.  VII,  facial  nerve; 
VIII,  acoustic  nerve;  a,  Bechterew's  nucleus;  D,  Deiters'  nucleus;  d,  dentate  nucleus; 
g,  nucleus  globosus;  p,  nucleus  embolif ormis ;  ra,  anterior  root  fibers;  sc,  sc^,  subcortical 
fibers  of  the  red  nucleus  and  of  the  thalamus  to  the  cortex;  t,  tegmental  nucleus. 
(Bechterew.) 

Treatment  of  Seasickness. — Seasickness  is  a  special  form  of  dis- 
turbance of  the  labyrinth.  As  the  stomach  has  little  or  nothing  to 
do  with  seasickness,  diet  has  little  or  no  effect  upon  this  malady,  and 
the  ocean  traveller  need  pay  no  more  attention  to  the  question  of  food 
than  that  dictated  by  common  sense.     Eat  one  must,  for  there  is 


232  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

nothing  worse  than  continued  retching  on  an  empty  stomach.  The 
oftener  one  vomits,  the  oftener  one  should  eat  or  take  hquid  food. 

If  one  is  predisposed  to  seasickness,  morning  walks  on  deck  before 
breakfast  should  be  dispensed  with.  One  should  try  to  breakfast 
immediately  upon  rising,  and  a  little  fruit  or  other  light  food  eaten 
before  rising  may  be  found  helpful.  What  one  eats  is  of  small  moment; 
the  great  thing  is  to  eat;  but  one  should  avoid  food  which  one  does  not 
like.  There  is  no  potency  in  any  particular  food  in  the  prevention  of 
seasickness. 

Nor  is  alcohol  of  any  use,  unless  enough  be  taken  to  anesthetize 
the  stomach.  Indeed  it  is  far  more  likely  to  prove  an  irritant,  espe- 
cially if  the  individual  be  unaccustomed  to  its  use.  The  much  vaunted 
value  of  champagne  is  a  myth  kept  alive  by  the  suggestion  of  dealers. 

One  should  not  go  to  dinner  until  it  is  just  about  to  be  served,  thus 
avoiding  the  discomfort  of  waiting  in  a  stuffy  and  perhaps  overheated 
dining  room.  When  the  meal  is  over  it  is  well  to  lie  down,  rather 
than  go  for  a  tramp  on  deck  in  the  hope  that  it  will  aid  digestion. 

Warm  clothing  and  wraps  should  be  taken  on  a  sea  voyage  even  in 
very  warm  weather.  Cold,  damp,  and  foggy  weather  is  apt  to  be 
met  with  on  the  ocean  at  any  time  of  the  year,  and  the  consequent 
chilliness,  added  to  that  of  an  unstable  vasomotor  control,  through  the 
labyrinth,  is  a  great  cause  of  discomfort,  which  may  be  removed  or 
alleviated  by  wearing  warm  garments. 

If  the  sea  be  at  all  rough  and  the  motion  of  the  vessel  appreciable, 
the  sensitive  traveller  should  lie  down  at  once,  as  it  is  easier  to  accustom 
one's  self  to  the  labyrinthine  hyperstimulation  in  a  recumbent  position, 
especially  if  one  adopts  the  position  in  which  the  motion  is  least  felt 
in  the  superior  canals,  i.  e.,  one  should  lie  down  as  flat  as  possible — 
semireclining  does  not  so  place  the  plane  of  the  semicircular  canal  as 
to  cause  the  least  possible  jflow  of  fluid  within  it.  One  flat  pillow  is  all 
that  one  should  use  since  half  sitting  up  is  as  bad  as  standing  up. 
Chairs  should  be  shifted,  if  possible,  according  to  the  pitch  or  roll  of 
the  vessel. 

As  soon  as  the  first  symptoms  of  seasickness  are  felt  the  patient 
should  lie  down,  if  possible,  on  deck.  As  eye  movements  aid  in  causing 
seasickness,  one  should  close  the  eyes  if  there  is  much  motion  of  the 
ship,  so  as  to  relieve  the  muscles  from  the  constant  adjustment  neces- 
sary in  watching  a  wavering  horizon,  and  in  very  bright  weather, 
colored  glasses  should  be  worn  to  subdue  the  glare. 

Reading  continuously  is  rather  to  be  avoided,  therefore  books 
should  be  chosen  which  will  allow  one  to  close  one's  eyes  and  meditate. 
Cards  or  other  games  which  divert  the  attention  are  very  helpful. 

In  making  choice  of  rooms,  one  should  give  preference  to  those  in 
the  middle  of  the  boat  where  the  motion  is  less  felt.  To  overcome  the 
smells  and  stuffiness  incident  to  ocean  travel,  one  should  keep  plenty 
of  air  circulating  in  one's  state  room,  unmindful  of  drafts,  which  are 
of  much  less  consequence  than  one  is  prone  to  think  them. 


DISEASES  OF  THE  HYPOGLOSSAL  NERVES 


233 


Eating  fruits  and  salads,  drinking  plenty  of  liquids,  and  occasion- 
ally taking  a  pill  of  aloes,  aloes  and  mastiche,  or  similar  laxative,  is 
generally  sufficient  to  counteract  the  constipation  which  is  a  frequent 
consequence  of  the  unusual  change  of  habits,  especially  when  one  eats 
very  little. 

The  headache  of  seasickness  is  best  combated  by  eating,  by  coffee, 
and  by  small  doses  of  bromids  and  phenacetin.  The  widely  used 
headache  mixtures  incorporating  caffein  and  antipyrin  in  the  elixir 
of  sodium  bromid  are  useful.  The  sodium  salt  of  veronal  in  doses  of 
from  8  to  10  grains,  given  by  rectum  in  suppository,  is  a  very  useful 
remedy  in  causing  sleep  and  in  relieving  excessive  irritability  of  the 
labyrinth. 


Table  of  Diffebential  Diagnosis  of  Labyrinthine  and  Cerebellar 
Disturbances. 


Tests. 


Spontaneous 
nystagmus 


Turning 
nystagmus 


Caloric 
nystagmus 
Compression 
Hearing 


Other  symptoms 


Treatment 


Circumscribed 
labyrinthitis. 


Fistula 
formation. 


May  be  absent 
on  sound  or 
diseased    side 

Produced  both 
sides  same  in- 
tensity 


Diminished   or 

not  present 

Present 

Hears  with  dif- 
ficulty or  deaf 

Vertigo  and 
nystagmus  on 
rapid  move- 
ment of  head, 
nausea  and 

Radical  opera- 
tion 


II. 
Diffuse  labyrinthitis. 


(a) 

Serous 

labyrinthitis. 


Mostly  on 

sound  side 

also  both 
sides 

Both  sides 
with  same  in- 
tensity 


Absent 

Absent 

Hears  with 
diificulty  or 
deaf 

Marked  ver- 
tigo, nausea, 
fever 


No  operation 


(6) 

Acute  purulent 
labyrinthitis 


Rotatory    and 
horizontal 
strong  on  the 
well  side 

Cannot  be 
tested  be 
cause  of  exces- 
sive reaction 

Absent 

Absent 
Deaf 


Vertigo,     nau- 
sea, vomiting 


Radical  and 
labyrinthine 
operation 


(0 
Chronic    puru- 
lent labyrinthi- 
tis. 


Weak  on  well 
side,  or  or 
both  sides 

Residual;  oe 
well  side,  nor- 
mal time;  on 
sick  side, 
shortened 

Absent 

Absent 
Deaf 


Slight  vertigo, 
also        slight 
equiUbrium 
disturbances 

Radical      and 
labyrinthine 
operation 


III. 

Diffuse     purulent     labyrinthitis 

and  cerebellar  abscess. 


Type  A. 


Strong  rotatory 
and  horizontal 
in  sick  side 


Type  B. 


Rotatory  and 
horizontal  on 
well  side 


Produced  both  sides  same  in- 
tensity 


Absent 


Absent 
Deaf 


Changeable  vertigo  and  equilib- 
rium disturbance;  finger  test 
positive,  eye  grounds  positive 


Radical,  laby- 
rinth and  cere- 
bellar opera- 
tion 


Radical  and 
labyrinth  op- 
eration.  If 
nystagmus 
con  tinues, 
cerebellar  op- 
eration 


DISEASES   OF   THE  SPINAL  ACCESSORY  AND  HYPOGLOSSAL 

NERVES. 

Accessorius. — The  spinal  accessory  nerve  innervates  the  sterno- 
cleidomastoid and  the  trapezius.  Branches  to  the  vagus  are  known. 
Its  cortical  origin  is  not  definitely  localized.  The  supranuclear  path- 
ways pass  through  the  internal  capsule  to  end  in  part  in  the  medulla 
near  the  olive  and  vagus  nuclei,  in  part  in  the  anterior  horns  of  the 
six  upper  cervical  "spinal"  segments.     The  combined  branch  passes 


234         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

by  way  of  the  jugular  foramen  to  its  muscle  distribution,  being  com- 
bined with  sympathetic  fibers  from  the  cervical  plexus. 

Clinical. — Cortical  disturbances  (first  motor  neuron)  cause  irregular 
and  spasmodic  actions.  These  are  seen  in  certain  epilepsies,  usually  con- 
ditioned by  cerebral  syphilis,  multiple  sclerosis,  or  other  brain  disorder. 

The  various  tics  (wry-neck,  etc.)  are  mostly  of  cortical,  i.  e.,  psychical 
origin.  They  represent  compulsion  neuroses  for  the  most  part.  (See 
Psy  choneuroses . ) 

Treatment  of  these  tics,  torticollis,  when  mental,  by  peripheral 
operations  is  folly. 

Nuclear  affections  of  the  accessorius  are  rare. 

Peripheral  palsies  are  not  infrequent  and  are  due  to  various  injuries, 
direct,  through  disease  of  the  base  of  the  skull,  syphilis,  osteitis.  They 
cause  degrees  of  loss  of  power  to  pull  the  face  to  one  side,  with  tendency 
to  contraction  of  the  opposite  side  (caput  obstipum).  Electrical 
changes,  R.  D.,  atrophy,  loss  of  reflexes,  are  present  in  the  nuclear  and 
peripheral  palsies,  but  are  absent  in  the  central  palsies  or  centrally 
induced  torticollis.  Trapezius  palsy  causes  the  characteristic  rotation 
of  the  scapula. 

Treatment  will  vary  with  cause.  It  can  only  be  emphasized  that 
the  surgical  treatment  of  spasmodic  torticollis,  which  in  the  over- 
whelming majority  of  cases  is  a  psychical  reaction,  usually  a  compul- 
sion neurosis,  is  useless.  Psychoanalysis  and  reeducation  have  been 
much  more  valuable. 

Hypoglossus. — ^The  hypoglossal  nerves,  twelfth  pair,  are  the  chief 
motor  nerves  of  the  tongue.  Through  collaterals  they  also  send 
motor  fibers  to  the  sternohyoid  and  sternothyroid  muscles.  The 
chief  physiological  functions  by  symmetrical  innervation  are  as 
follows:  The  genioglossus  muscle  moves  the  tongue  forward  and 
down,  the  hypoglossus  muscle  moves  the  tongue  back  and  up.  The 
styloglossus  moves  the  base  of  the  tongue  up  and  back.  In  a  sym- 
metrical innervation — that  is,  loss  on  one  side — the  combined  action 
of  these  three  muscles  causes  the  tongue  to  deviate  in  toto  to  the 
paralyzed  side. 

Affection  of  the  longitudinal  muscle  by  symmetrical  innervation 
causes  shortening  of  the  tongue,  either  pulling  the  top  of  the  tongue 
up  or  down.  By  asymmetrical  innervation  the  anterior  portion  of  the 
tongue  is  pushed  to  the  paralyzed  side.  Any  loss  of  the  function  of 
the  branch  which  innervates  the  transverse  muscles  brings  about  a 
narrowing  of  the  tongue,  whereas  symmetrical  affection  of  the  vertical 
muscles  produces  a  flattening  of  the  tongue.  The  geniohyoid  raises 
the  hyoid  bone,  pulling  it  forward  when  the  lower  jaw  is  fixed,  or  pulls 
the  lower  jaw  down.  When  the  hyoid  bone  is  fixed  the  sternohyoid 
and  the  thyreohyoid  pull  on  the  hyoid  bone. 

The  nucleus  of  the  hypoglossus  lies  in  the  lower  two-thirds  of  the 
medulla,  stretching  as  far  down  as  the  pyramidal  crossing,  ventrally 
from  the  central  canal  to  the  midline.     At  least  ten  to  fifteen  root 


DISEASES  OF  THE  HYPOGLOSSAL  NERVES 


235 


bundles  pass  from  the  hypoglossal  nuclei  between  the  pyramidal  tracts 
and  the  olive,  and  join  together  for  a  short  distance  within  the  hypo- 
glossal canal,  at  the  orifice  of  which  the  hypoglossal  vein,  which  is  in 


Fig.  119.— Pathways  of  the  taste  fibers.  I,  ophthalmic  branch  of  V,  II,  maxiUary 
branch;  III,  mandibular  branch;  eg,  cortical  taste  area;  fg,  central  ascending  taste 
fibers  in  median  lemniscus;  fg,  subcortical  paths;  Gg,  geniculate;  Gsp,  jugular  and 
petrosal  ganglia  of  the  glossopharyngeus;  ta,  central  ascending  fibers  of  trigemmus  m 
median  lemniscus;  ta\  subcortical  connections  of  the  thalamus  with  the  inferior  posterior 
central  gyrus.    (Bechterew.) 


236         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

connection  with  the  occipital  sinus,  surrounds  it.  The  canal  is  narrow 
and  short — less  than  half  an  inch — lying  close  to  the  occipito  atlantic 
articulation,  at  a  place  where  fracture  of  the  base  of  the  skull  is  very 
apt  to  affect  it. 

At  its  exit  from  the  skull  the  hypoglossal  lies  median  to  and  dorsal 
of  the  vagus  and  of  the  internal  jugular  vein,  proceeds  laterally,  passes 
beneath  the  stylohyoid  muscle  and  the  posterior  belly  of  the  kigastric, 
splits  into  its  various  branches,  and  innervates  the  muscles  already 
mentioned. 

Anastomoses  with  the  ganglion  nodosum,  superior  cervical  ganglion, 
the  lingual  and  first  and  second  cervical  nerves  take  place.  A  special 
branch,  the  ansa  hypoglossal  is  formed  by  anastomoses  of  the  descend- 
ing branch  and  the  hypoglossal,  and  branches  from  the  second  and 
third  cervical  nerves. 

The  supranuclear  pathways  are  followed  with  considerable  difficulty. 
In  the  cerebral  peduncles  they  lie  in  the  center  somewhat  more  median 
than  the  facial;  within  the  internal  capsule  they  lie  at  the  knee.  The 
cortical  stations  lie  in  the  lower  portion  of  the  central  convolutions. 
The  supranuclear  pathways  decussate  freely. 

Peripheral  lesions  of  the  hypoglossal  are  the  result,  usually,  of 
mechanical  causes,  resulting  either  from  fracture  of  the  base  of  the  skull, 
from  tumors,  direct  injury  or  tuberculosis,  or  dislocation  of  the  upper 
cervical  vertebrae.  Cerebrospinal  syphilis,  particularly  of  long  stand- 
ing, is  a  not  infrequent  cause  of  peripheral  palsies,  while  poisoning  from 
lead,  arsenic,  alcohol,  carbon  monoxide  may  cause  peripheral  lesions. 

Nuclear  and  supranuclear  affections  of  the  hypoglossal  are  due 
to  hemorrhage  within  the  medulla.  Poliomyelitis,  tumors,  syphilis, 
and  multiple  sclerosis — these  are  the  most  frequent  cause  of  nuclear 
or  supranuclear  lesions  of  these  nerves. 

Clinical. — The  most  frequent  lesion  of  the  hypoglossal  is  unilateral. 
There  is  atony  of  the  longitudinal  muscles  of  the  paralyzed  side,  and 
when  the  tongue  lies  quiet  in  the  mouth  its  apex  deviates  slightly  to 
the  non-paralyzed  side.  The  base  of  the  tongue  usually  rises  higher 
on  the  paralyzed  side  than  on  the  sound  side  as  a  result  of  atony  of 
the  hypoglossal  muscle. 

Movements  of  the  tongue  are  diminished;  it  becomes  difficult  to 
remove  food  which  lies  between  the  teeth  and  the  cheek,  and  it  becomes 
difficult  for  the  patient  to  direct  the  tongue  to  the  teeth  on  the  par- 
alyzed side.  On  thrusting  the  tongue  out  it  deviates  to  the  sound  side. 
Speech  disturbances  are  present,  especially  the  labials  and  Unguals. 

In  long-standing  disease  atrophies  develop  with  fibrillary  twitchings 
and  tremors  and  pronounced  irregularities,  and  electrical  stimulation 
shows  reaction  of  degeneration. 

In  bilateral  paralyses  speech  disturbances  are  very  marked.  Chew- 
ing and  swallowing  are  rendered  difficult,  and  the  movements  of  the 
tongue  are  markedly  diminished  in  all  directions. 

In  hysterical  tongue  paralyses,  which  are  by  no  means  uncommon, 


DISEASES  OF  THE  HYPOGLOSSAL  NERVES 


237 


resistance  to  passive  motion  of  the  tongue  is  seen.    There  are  no  elec- 
trical changes  and  speech  disorder  is  apt  to  be  obvious. 

In  nuclear  palsies,  atrophy  and  fibrillary  twitching  are  marked,  the 
speech  disturbances  are  pronounced,  the  chief  characteristic  being 
what  is  termed  "hot-potato  speech."  The  patient  speaks  as  though 
he  had  a  hot  morsel  in  his  mouth.  Reaction  of  degeneration  is  also 
present.  Supranuclear  palsies,  such  as  occur  in  hemiplegia,  involve 
the  muscles  as  a  whole,  cause  deviation  of  the  tongue  to  the  paralyzed 
side,  and  other  signs  of  hemiplegia  are  present.  Isolated  cortical 
spasm  of  the  hypoglossal  may  be  present. 


Fig.  120. — Atrophy  of  right  half  of  tongue.     Cerebral  syphilis  and  injury. 

Psychogenetic  hypoglossal  disturbances  are  by  no  means  rare. 
These  consist  of  tongue  tics,  of  constant  tongue  movements,  such 
as  are  seen  in  hysterias,  in  patients  with  dementia  precox,  compulsion 
neurosis,  and  in  various  paranoid  types  of  thinking. 

Treatment  of  hypoglossal  disturbances  varies  according  to  the  cause 
and  is  usually  purely  symptomatic. 

Speech  Disturbances. — Human  expression,  articulatory,  mimetic, 
tactile,  or  by  writing,  is  a  highly  complicated  mechanism.  It  includes 
all  of  those  movements  resulting  from  optical,  auditory,  or  tactile 
contacts  by  which  communication  between  individuals  is  brought 
about  for  social  purposes.  Language  as  it  fully  develops  is,  therefore, 
a  tool  with  which  one  may  cut  into  reality  and  utilize  the  facts  of 


238 


SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 


nature  for  ultimate  adaptation.  Thus,  all  speech  disturbances  may  be 
divided  into  those  in  which  the  receptive  (sensory)  side  of  the  pathways 
are  involved  and  into  those  in  which  the  productive  (or  purely  motor) 
part  of  the  arc  is  implicated. 

On  the  sensory  side  one  finds  the  gradual  accumulation  of  experience, 
chiefly  with  auditory  stimuli  (with  the  gradual  evolution  of  speech), 
symbols  (language)  which  stand  in  the  developing  psyche  for  the 
images  of  things,  ideas  or  feeling  values.     Thought  is  symbolic  action. 

Optical  stimuli — objects,  signs,  various  glyphs,  words,  letters — 
unite  to  more  complex  types  of  expression  in  writing  (psychically 
developed  to  conquer  spatial  limitations).  While  tactile  stimuli 
are  an  integral  part  of  language  from  the  more  restricted  side  of 
tactile  reading  of  blind  and  tactile  speech  of  the  deaf  and  dumb  to 


Fig.  121.- — ^Scheme  of  speech  connections.     (Veraguth.) 

the  sensory  stimuli  of  the  movements  of  the  muscles  and  tongue  and 
lips  in  speech.  A  complete  analysis  of  the  great  complex  of  sensory 
factors  which  ultimately  find  outlet  in  speech  symbolism  with  its 
infinite  psychical  implication  is  not  possible  here.  It  would  involve 
the  entire  problem  of  the  evolution  of  civilization  and  culture.^ 

The  productive  side  of  the  speech  mechanism  is  less  complicated. 
Originally  showing  itself  in  the  child  as  a  noisy  symbol  of  crying,  there 
is  gradually  shaped  by  the  libido  more  and  more  accurate  sounds  of 
expression  to  meet  the  needs  of  hunger  and  of  love.  Grunts,  laughs, 
gurgles  evolve  into  more  precise  formulations,  until  the  rich  symboli- 
zations  of  speech  are  gained,  with  all  their  advantages  of  precision  and 
efficiency.  Here  the  muscles  of  the  mouth,  lips,  larynx,  chest,  abdo- 
men, arms,  and  pelvis  all  come  into  a  gradually  refining  and  orderly 
series  of  coordinated  activities. 


1  Gutzmann.     Sprach-Storungen.     Various  monographs  on  Aphasia. 


DISORDERS  OF  SPEECH  239 

Many  schemes  have  been  devised  to  set  forth  graphically  some  of 
the  phases  of  these  speech  disturbances.  One  of  Veraguth's  is  here 
utilized. 

Here  1  represents  the  incoming  auditory  stimuli  (tone,  sound,  words) 
with  their  more  or  less  sharply  defined  sensorial  perceptions  gained 
gradually  through  experience.  They  constitute  in  their  totality  the 
various  heard  components  of  speech  and  are  constituents  of  organic, 
auditory  memories.  The  circle  I  represents  such  a  psychophysio- 
logical combination,  rather  than  an  anatomical  hearing  center,  which 
latter  is  roughly  outlined  in  the  first  and  second  temporal  convolutions. 

Pathway  2  represents  the  optic  as  well  as  the  tactile,  and  kinesthetic 
neuron  chain  which  convey  to  the  brain  centrals  graphic  symbols 
(pictures,  diagrams,  graphs,  letters,  etc.).  The  general  assembly  place 
of  these  is  represented  by  the  circle.  It  represents  not  an  anatomically 
circumscribed  area,  but  rather  a  functional  capacity,  but  not  unrelated 
to  an  optical  sensory  area  in  the  cuneus  and  precuneus  of  the  occipital 
lobes. 

Outgoing  pathways  3  and  4  represent  the  motor  side  of  the  arcs 
of  expression  by  all  those  motions  by  which  the  act  of  articulation 
with  infinite  variation,  shades,  and  nuances,  and  those  of  graphic 
representation  are  carried  out.  Both  pathways  are  related  to  cortical, 
bulbar,  spinal  localizations,  which  make  functional  unity  possible 
as  speech  and  writing  (in  widest  sense).  These  are  symbolized  by 
circles  III  and  IV.  All  of  these  are  brought  together  in  an  enlarged 
concept  (circle  V),  which  symbolizes  the  heard,  read,  spoken,  or  written 
mode  of  expression  (words,  acts,  representation,  mimic,  etc.). 

The  lines  which  bind  these  various  centers  represent,  therefore,  a 
series  of  possibilities.  Whereas  an  anatomical  substratum  underlies 
these  possibilities,  no  attempt  will  be  made  to  represent  them  here. 

1.  Repetition  of  words  without  comprehension — Pathway  1,  5,  3. 

2.  Reading  aloud  without  comprehension — Pathway  2,  7,  3. 

3.  Writing  to  dictation — Pathway  1,  6,  4. 

4.  Writing  to  dictation  without  sense — 2,  8,  4. 

5.  When  heard  word  is  comprehended — 1,  9. 

6.  When  read  word  is  comprehended — 2,  12, 

7.  Spontaneous  speech  of  an  idea — 10,  3. 

8.  Spontaneous  graphic  expression  of  an  idea — 11,  4. 

9.  When  heard  word  is  comprehended  and  reproduced  by  speech — 

1,  9,  10,  3. 

10.  When  heard  word  is  comprehended  and  reproduced  graphic- 
ally—1,  9,  11,  4. 

11.  When  read  word  is  comprehended  and  reproduced  by  speech — 

2,  12,  10,  3. 

12.  When  read  word  is  comprehended  and  reproduced  graphically — 
2,  12,  11,  4. 

The  scheme  also  attempts  to  show  an  internal  and  external  speech. 
At  the  present  time  exact  correlation  between  all  types  of  speech 


240  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

disturbance  and  definite  pathways  cannot  be  made.  But  in  the  main 
certain  broad  facts  have  accumulated  to  permit  certain  fairly  exact 
generalizations.  In  the  first  place  the  general  speech  mechanisms  are 
located  predominantly  in  the  left  hemisphere  in  right-handed  individ- 
uals. In  the  left-handed  the  localization  is  predominantly  in  the  right 
hemisphere.  Ambidextrous  brains  are  known  and  educable  opposite 
speech  areas  are  known. 

While  one  speaks  of  speech  areas,  auditory  (temporal)  and  optic 
(occipital),  motor  (Broca's  convolution)  and  attempts  to  localize 
them,  the  fact  is  that  the  architecture  of  the  brain  is  so  complex,  the 
pathways  utilized  in  the  speech  mechanisms  so  widespread,  that  it 
is  better  to  speak  of  aphasia  areas.  These  are  areas  of  special  predilec- 
tion for  the  occurrence  of  speech  disturbances  which  are  more  or  less 
stereotyped.  There  are  areas  which  are  supplied  more  particularly 
by  the  1,  2,  3,  4  branches  of  the  Sylvian  artery,  distributed  to  the  third 

Sulcus  centralis 


/ 

t  ossa   Sylvii 
Fig.  122. — -The  aphasia  regions  in  the  left  hemisphere.     (Veraguth.) 

frontal  convolution  and  operculum  of  the  central  convolution,  Broca's 
area  (motor  speech  aphasias),  the  insula  (Heschl  convolution),  the 
posterior  part  of  the  first  temporal,  auditory  centers,  amnesic  aphasia, 
and  the  angular  gyrus  (Cuneus,  optic  alexias). 

These  areas  are  well  shown  in  v.  Monakow's  diagram  here  repro- 
duced. 

Clinical  Forms. — ^These  may  be  subdivided  into  external  and  internal 
speech  disturbances. 

I.  Deafness  brings  about  a  special  form  of  speech  disturbance 
(deaf  mutism).  Even  though  the  speech  apparatus  be  intact,  it 
lacks  the  dynamic  stimuli  to  be  utilized.  When  speech  is  acquired, 
it  has  a  peculiar  monotonous  quality.  Certain  forms  of  mutism 
from  faulty  hearing  are  to  be  distinguished. 

II.  Dysarthrias. — Used  in  a  broad  sense,  these  include  disturbances 
in  speech  due  to  defect  in  the  productive  pathway. 


APHASIAS 


241 


(a)  They  may  be  of  purely  psychogenic  origin,  i.  e.,  compulsive 
ideas,  hysterical  conversions,  psychotic  symbol  distortion,  such  as 
are  evidenced  by  stammering,  stuttering,  hysterical  speech,  katatonic 
speech. 

(6)  Peripheral  motor  palsies;    facial,  palate  (rhinolalia). 

(c)  Bulbar  palsies  (mouthful  speech),  as  seen  in  progressive  muscular 
atrophy  (Aran-Duchenne  type,  often  syphilitic)  in  amyotrophic 
lateral  sclerosis,  also  at  times  of  same  etiology,  in  acute  poliomyelitis, 
in  multiple  sclerosis,  syringomyelia,  in  tumors  of  the  medulla  and 
pons,  and  in  general  paresis.    In  this  latter  case  almost  specific  speech 

Arm  Centre 


Splenium Corpus  Callosum 


Genu  Corpus  Callosum 


Fronia 
Lobe 


Occipital 
Lobe 


Fj  op 

Motor  Aphasia 


Ti 

Sensory  Aphasia 


Fig.  123.— Scheme  of  the  chief  areas  and  pathways  involved  in  aphasic  disturbances.  . 
Nos.  3,  4,  5,  6,  7,  sensory  pathways;  Nos.  1,  2,  8,  9,  10,  11,  motor  pathways.  Str.,  corpus 
striatum;  Li,  lenticular  nucleus;  Tho,  optic  thalamus;  op.,  operculum;  1,  path  from  left 
temporal  to  right  temporal  by  way  of  the  corpus  callosum;  2,  path  from  occipital 
lobe  to  the  arm  region;  3,  path  from  the  arm  region  to  the  internal  capsule  and  per- 
ipherally; 4,  path  from  temporal  lobe  to  arm  region;  5,  paths  from  the  prefrontals  to 
Fs]  6,  connecting  path  with  the  right  hemisphere  by  means  of  the  corpus  callosum; 
7,  paths  from  Fs  through  the  internal  capsule  downward;  8,  connecting  paths  from 
Fz  to  Ti,  operating  both  ways;  9,  path  from  the  internal  geniculate  to  T';  10,  connec- 
tions between  angular  and  supramarginal  gyri  and  Ti;  11,  path  between  occipital  and 
Ti.    (Veraguth  after  v.  Monakow.) 


disturbances  appear  which  are  described  under  the  chapter  on  Syphilis 
of  the  Nervous  System, 

(d)  In  disorders  of  the  static  equilibrium  mechanism,  as  in  chorea, 
paralysis  agitans,  multiple  sclerosis,  one  finds  incoordination  or 
scanning,  jerky,  or  monotonous  speech. 

The  disturbances  of  inner  speech  are  termed  ayhasias.  They  are 
here  divided  into  {A)  Receptor,  and  {B)  Productive,  which  are  prac- 
tically synonymous  with  sensory  and  motor. 

Aphasias. — Although  the  separation  of  all  aphasias  into  two  groups, 
receptive  and  productive,  is  theoretically  possible,  practically  this  is 
not  the  case.  The  actual  clinical  pictures  seen  vary  enormously. 
16 


242  SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

So  great  is  this  variation  that  a  text-book  description  of  aphasia  is 
almost  impossible.  It  may  be  accepted,  however,  that  lesions  in  the 
frontal  section  of  the  speech  area,  which  includes  the  anterior  portion 
of  the  island  of  Reil  (Heschl's  Convolution)  will  occasion  the  motor 
speech  types  of  aphasia,  here  called  productive;  whereas  lesions  in 
the  temporal,  parietal,  and  occipital  regions  will  give  rise  to  sensory, 
or  receptive,  aphasias.     (See  Fig.  122.) 

Without  attempting  to  go  into  minute  details  the  lesion  in  the 
motor  speech  area  gives  rise  to  a  loss  of  the  motor  components  of 
words,  that  is  to  say,  the  patient  is  unable  to  assemble  his  motor 
memory  images  of  words.  In  motor  aphasia  then  he  is  more  or  less 
dumb,  and  is  able  to  say  only  a  few  words  or  parts  of  words  or  frag- 
ments of  sentences.  The  patient  is  unable  to  repeat  words  on  request 
and  cannot  read  aloud  spontaneously.  For  the  most  part  he  is  also 
unable  to  write  spontaneously  or  to  write  to  dictation,  but  he  may 
be  able  to  copy.  This  is  the  usual  picture  of  what  is  known  as  complete 
motor  aphasia. 

In  the  contrasting  picture  of  complete  sensory  aphasia  there  is  no 
difficulty  in  speaking  per  se,  but  the  patient  cannot  talk  comprehend- 
ingly  and  is  apt  to  babble.  He  hears  sounds  and  tones  but  does  not 
know  that  they  are  words  and  cannot  use  them  in  their  proper  way. 
He  does  not  recall  the  meaning  of  these  sounds,  either  as  to  their 
letters,  nor  in  the  letter  combinations,  as  syllables  or  words.  If  this 
sensory  aphasia  involves  chiefly  his  memory  of  sounds  he  is  word- 
deaf  and  suffers  from  auditory  sensory  aphasia.  He  may  also  make 
mistakes  in  writing  just  as  he  does  in  speaking;  and  paraphasia,  or 
its  severe  grades,  known  as  jargon  aphasia,  in  speech  is  paralleled  by 
similar  mistakes  in  writing,  paragraphia.  The  patient  may  also  make 
foolish  mistakes  in  reading,  paralexia,  or  at  times  he  is  unable  to 
read  at  all,  alexia.  If  he  does  not  understand  what  he  sees  one  speaks 
of  him  as  being  word-blind. 

These  are  the  two  chief  forms  of  aphasia. 

A  total  aphasia,  which  frequently  follows  a  rupture  of  the  Sylvian 
artery,  involving  both  the  frontal  and  the  temporoparietal  regions 
of  the  speech  domain  is  even  more  frequent  than  motor  aphasia  or 
sensory  aphasia.  Most  aphasias  partake  of  this  dual  nature.  In  the 
majority  of  cases  the  motor  defect  masks  the  sensory  one. 

In  addition  to  these  main  divisions,  partial  aphasias  have  been 
described.  A  complete  summary  of  these  would  fill  a  large  volume. 
A  few  of  the  more  characteristic,  however,  are  worth  picking  out. 
Students  should  recall,  however,  that  the  aphasia  problem  is  highly 
complicated,  and  every  case  presents  marked  variations. 

Isolated  hemorrhages  or  tumors  which  involve  the  island  of  Reil, 
give  rise  to  what  is  known  as  an  island  aphasia.  Here  association 
fibers  between  the  auditory  and  motor-memory  centers  are  cut  off. 
The  clinical  symptoms  closely  resemble  motor  aphasia.  Spontaneous 
speech  is  much  impaired  and  the  patients  are  better  able  to  repeat  on 


APHASIAS 


243 


dictation.  There  is  apt  to  be  a  certain  amount  of  paraphasia,  patients 
talking  a  moreor  less  senseless  jargon.  Paragraphia  is  alsousually  found. 
Pure  word-deafness  is  a  type  in  which  the  patient  cannot  under- 
stand spoken  speech.  He  therefore  cannot  write  to  dictation  nor  can 
he  repeat  the  words  spoken  to  him;  on  the  other  hand  he  is  able  to 
speak  spontaneously,  can  read  aloud,  can  write  and  can  copy.  He 
has  no  paragraphia,  paralexia  or  paraphasia.  He  is  simply  word- 
deaf,  being  unable  to  distinguish  word  sounds  from  other  sounds. 
He  behaves  a  good  deal  like  a  patient  who  seems  to  be  deaf,  and  in 
testing  for  this  type  of  aphasia  it  is  important  to  exclude  deafness. 
In  this  form  of  aphasia  the  lesion  is  usually  located  beneath  the  cortex 
of  the  first  and  second  temporal  convolutions. 


General  Scheme  op  Different  Aphasia  Types.     (Eichorst.) 


Type  of  aphasia. 


1.  Cortical  motor  apha- 
sia. (Word-dunab- 
ness.) 


2.   Transcortical    motor 
aphasia. 


3.  Subcortical       motor 
aphasia. 


4.   Cortical  sensoiy 

aphasia.  (Com- 
plete word-deaf- 
ness.) 


5.   Transcortical       sen- 
sory aphasia. 


6.   Subcortical     sensory 
aphasia.  (Pure 

word-deafness.) 


7.   Conduction  aphasia. 


Location  of  lesion. 


Broca's  center,  pos- 
terior two-thirds 
of  the  third  left 
convolution,  pars 
triangularis  and 
operculum. 

Hypothetical.  Cut- 
ting of  paths  be- 
tween (1)  with 
ideational  center 
in  frontal  region. 

Connection  between 
Broca's  center 
and  the  bulbar 
nuclei  of  the  mus- 
cles of  speech. 

Wernicke's  center; 
posterior  third  of 
the  superior  tem- 
poral gyrus  and 
gyrus  supramar- 
ginalis. 

Connections  be- 
tween 4  and  idea- 
tional center, 
otherwise  as  in  2. 


Connections  of 

Wernicke's  center 
mostly  lesions  in 
the  left  temporal 
lobes  or  of  corpus 
callosum  between 
the  temporal  lobes. 

Lesions  in  cortex 
and  gray  matter 
of  the  island  of 
Reil. 


Loss  of  function. 


Spontaneous  speech. 
Repetition  of 

words.  Reading, 
writing  (to  dic- 
tation or  spon- 
taneous) .  Ability 
to  repeat  syllables. 

Spontaneous  speech. 
Spontaneous  writ- 
ing. 


Spontaneous  speech. 
Repeat  spoken 
words.  Reading 
aloud. 

Comprehension  of 
speech.  Repeat 
spoken  word. 

Reading.       Writ- 
ing to  dictation. 

Comprehension  of 
speech.  Compre- 
hension of  writ- 
ing. 


Comprehension  of 
speech.  Repeat- 
ing of  words. 
Copying  to  dic- 
tation. 


Repeating  words. 
Reading  aloud. 
Writing  to  dicta- 
tion. 


Intact  function. 


Comprehension  of 
words.  Compre- 
hension of  writing. 
Copying. 


As  1,  also  can  repeat 
spoken  words. 

Write  to  dictation. 
Read  aloud. 

Understands  speech. 
Understands  writ- 
ing. Spontaneous 
writing.  Copy  and 
copy  to  dictation. 

Spontaneous  speech. 
Spontaneous  writ- 
ing. Copying.  Par- 
aphasia. Para- 
graphia. Senseless 
copying. 

Repeat  words,  and 
read  aloud  with- 
out comprehen- 
sion. Copy  to  dic- 
tation without 
comprehension . 

Spontaneous  speech. 
Reading  aloud. 
Comprehension  of 
writing.  Spontan- 
eous copying  writ- 
ing. 

Paraphasia.  Para- 
graphia. Compre- 
hension of  writing. 
Comprehension  of 
speech.     Copying. 


244         SENSORI-MOTOR  NEUROLOGY— CRANIAL  NERVES 

In  addition  to  these  forms  there  are  described  pure,  or  subcortical, 
motor  aphasia,  and  pure,  or  subcortical  word-deafness.  Cortical 
motor  aphasia,  cortical  sensory  aphasia  and  isolated  alexias,  agraphias, 
etc.,  these  must  be  looked  for  in  larger  works  on  the  subject,  already 
referred  to. 

A  short  summary  of  these  characteristic  forms  is  given  on  page  243. 

Writing  Disturbances. — A  great  variety  of  disturbances  in  writing, 
quite  analogous  to  speech  disturbances,  are  known.  Thus,  writer's 
cramps  are  analogous  to  stuttering  and  stammering.  They  are 
probably  psychogenic  in  origin.  In  the  psychoses,  very  characteristic 
writing  features  and  failures  are  present.  The  paretic  may  write  just 
as  he  speaks,  slurring,  leaving  out  syllables  or  words,  etc.  The  kata- 
tonic  may  show  stilted  writing  just  as  he  shows  a  stilted  affected  speech. 
The  haste  of  the  manic  is  seen  in  writing  as  in  speech.  In  time  a  true 
science  of  chirograph}^  may  be  built  up  on  a  psychical  basis,  just  as  a 
true  science  of  phonetics. 

Paralysis  agitans,  multiple  sclerosis,  shows  analogous  features 
in  writing  and  in  speech. 


CHAPTER  VL 

AFFECTIONS  OF  THE  PERIPHERAL  NERVES:  SENSORY 

AND  MOTOR. 

NEURALGIAS. 

TriE  most  characteristic  feature  of  disordered  peripheral  sensory 
nerves  is  pain.  In  the  presence  of  pain  alone,  one  speaks  of  neuralgia; 
pain  with  trophic  disorders,  tender  nerve  trunks  and  altered  muscular 
function  when  peripheral  is  usually  termed  neuritis;  radiculitis  if  in 
the  roots  or  the  plexus,  whereas  pain  associated  with  peculiar  skin 
eruptions  due  to  ganglionic  root  involvement  is  called  herpes  zoster 
or  zona.  The  psychic  pain  of  hysteria,  and  the  central  pains  of 
thalamic  lesions  are  not  now  considered. 

The  boundaries  between  these  affections  are  largely  artificial.  Thus 
a  very  mild  neuritis  presents  only  its  neuralgic  features;  and  a  zona 
may  be  so  slight  as  to  cause  no  eruption.  From  a  clinical  standpoint 
separation  of  these  processes  may  be  impossible.  It  is  not  always 
necessary.  Pathologically  speaking,  one  locates  the  lesion  of  zoster 
in  the  sensory  ganglion,  yet  tic  douloureux — or  trigeminal  neuralgia — 
is  preeminently  a  disease  of  the  sensory  ganglion,  the  Gasserian,  and 
yet  there  is  rarely  any  zoster  eruption.  One  invokes  the  etiological 
factor  of  an  acute  infection  element  in  herpes  zoster  yet  there  are 
zoster  cases  due  to  other  than  bacterial  causes.  Sciatic  neuralgia  or 
sciatic  neuritis  indicates  a  fluctuating  point  in  the  causation  and 
severity  of  sciatic  pains.  The  differentiation  between  a  radiculitis 
and  a  neuritis  is  often  solely  a  question  of  terminology.         *^ 

Too  much  weight,  therefore,  is  not  to  be  laid  upon  the  classifications 
given.  For  practical  purposes  these  affections  are  treated  under 
three  heads,  but  their  fluctuating  separations  should  not  be  forgotten. 
It  is  useless  to  call  neuralgia  a  functional  disorder.  This  is  tantamount 
to  saying  it  is  a  disorder  of  unknown  causation,  and  hence  serves  as 
a  cloak  for  careless  investigation. 

Like  many  other  conditions  in  nature,  these  affections,  when  seen  in 
an  accentuated  and  pure  form,  for  practical  purposes,  represent  different 
entities,  yet  the  partial  and  intermediary  forms  are  so  many  that  the 
description  of  the  clear  cut,  classic  types  does  not  do  justice  to  the 
forms  found  in  nature. 

Definition. — A  painful  affection  of  the  nerve  trunk  or  its  branches, 
characterized  by  remittent  or  intermittent  flashes  of  acute  pain,  with 
free  intervals,  not  usually  accompanied  by  trophic  disturbances  of  the 
muscles,  unless  its  severity  limits  the  activities  of  an  organ,  occasionally 
associated  with  painful  nerve  trunks  and  with  disturbances  in  the  skin 
structures. 


246  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Neuralgias  are  but  the  expression  of  many  diverse  lesions  which 
may  involve  the  body  in  general,  as  toxic  and  infectious  states,  a  nerve 
trunk  itself,  the  sensory  ganglia,  contiguous  structures,  or  they  may 
be  the  reflex  expression  of  a  disorder  in  a  viscus  remote  from  the 
site  of  the  pain.  They  may  be  of  purely  psychogenic  origin,  mostly 
hysterical  conversions,  occasionally  delusional  projections.  Neural- 
gia therefore  is  to  be  considered  solely  as  a  symptom,  a  syndrome, 
or  a  purely  reflex  condition.     There  are  no  idiopathic  neuralgias. 

Etiology. — An  extraordinarily  wide  range  of  causative  factors  may 
determine  mild  or  severe  neuralgias  in  very  diverse  regions  of  the  body. 
The  most  frequent  causative  factors  are : 

(a)  Anemias  due  to  hemorrhages,  chlorosis,  pernicious  anemia,  and 
parasites. 

(6)  Toxins  of  exogenous  origin,  inorganic  and  organic,  or  purely 
endogenous  toxins:  thus  poisoning  by  lead,  mercury,  arsenic,  and 
copper.  Alcohol  and  tobacco  are  frequent  causes.  Morphinism 
causes  neuralgia  as  an  abstinence  symptom.  The  toxins  of  many 
infectious  disorders  are  especially  prone  to  bring  about  neuralgias. 
Tonsillitis  and  malaria  are  examples.  Typhoid  fever,  measles,  gon- 
orrhea, possibly  syphilis,  and  streptococcic  infections  are  frequently 
accompanied  by  neuralgias.  The  endogenous  toxemias  of  diabetes 
and  latent  nephritis  are  further  examples. 

(c)  Inflammation  of  the  sensory  ganglia,  which  may  be  either  of 
infectious  or  non-infectious  nature,  gives  rise  to  some  of  the  severest 
forms,  as  seen  in  herpes  zoster.  These  zosters  occur  from  involvement 
of  any  ganglion,  from  the  uppermost  to  those  furthest  caudad.  They 
are  usually  dealt  with  in  books  on  dermatology,  but  they  are  essential 
nervous  disorders.  Ganglion  involvements  of  non-infectious  types 
give  rise  to  neuralgias,  such  as  tic  douloureux,  while  tumors  of  the 
sensory  ganglia  may  condition  persistent  and  obstinate  neuralgias  in 
the  affected  sensory  nerves. 

(d)  Involvements  of  the  nerve  trunks  themselves,  either  by  mild 
neuritic  processes,  perineuritis,  pressure  from  anatomical  structures, 
pressure  from  lesions,  cuts,  bullets,  wounds,  tears,  tumors,  aneurism, 
exostoses,  fractures,  or  displacements  may  cause  severe  neuralgias. 
If  the  nerves  degenerate  neuritis  results. 

(e)  Reflex  neuralgias  are  numerous  and  puzzling.  Pulmonary 
cardiac,  gastric,  hepatic,  renal,  ureteric,  intestinal,  vesical,  uterine, 
ovarian,  prostatic,  testicular,  and  affections  of  other  viscera  give  rise  to 
herpetic  eruptions,  with  painful,  sensitive  skin  areas  and  neuralgias;  in 
many  instances  the  neuralgia  is  not  accompanied  by  herpes.  Head's^ 
complete  analysis  of  this  class  of  cases  is  of  paramount  importance. 
Thus  a  persistent  sciatica  may  be  the  reflex  of  a  prostatic  disturbance. 
An  anemic  woman  may  not  suffer  from  pain,  but  on  menstruation  her 
referred  neuralgic  pains  may  become  very  severe  over  the  tenth  dorsal 

1  Brain,  xvi,  1;  xvii,  339;  xix,  153. 


NEURALGIAS  247 

nerve,  and  pain  and  tenderness  are  frequent  over  the  areas  of  the  sixth 
dorsal  (heart),  seventh  dorsal  (stomach),  and  there  may  be  occipital 
and  midorbital  neuralgia  (Head).     (See  Figs.  124  and  125.) 

(/)  Organic  disease  of  the  nervous  system.  General  paresis,  tabes 
dorsalis,  spinal  or  cerebral  disease,  thalamus  disease,  syphilitic  menin- 
gomyelitis,  etc.,  are  often  accompanied  by  neuralgic  pains. 

(g)  Constitutional  Factors:  The  arthritic,  gouty,  rheumatic,  and 
scrofulous  may  be  said  to  be  predisposed.  Unknown  factors  thought 
to  be  related  to  atmospheric  pressure,  humidity,  high  electrical  tension, 
etc.,  play  a  role  in  many  of  these  cases. 

(h)  Chronic  vascular  disease,  and  especially  arteriosclerosis,  is  a 
frequent  cause,  particularly  in  the  aged,  the  senile,  and  the  presenile. 
Syphilitic  vascular  disease  is  a  cause. 

{i)  Exposure  to  cold  is  an  important  factor.  It  is  not  certain  that 
all  neuralgias  caused  by  cold  are  not  really  mild  types  of  neuritis  or 
perineuritis;  discussion  of  the  distinction  is  fruitless.  The  older 
writers  found  colds  a  predisposing  cause  in  from  25  to  40  per  cent,  of 
the  cases.     In  damp,  cold  countries  this  is  particularly  noticeable. 

(j)  Psychogenic  Factors:  These  play  a  large  part  in  practical 
medicine  in  determining  neuralgic  pains,  so-called. 

Symptoms. — Pain  is  the  main  factor  in  neuralgia.  For  the  most 
part  it  is  the  only  expression  of  the  nerve  disturbance.  The  character 
of  the  pain  varies  considerably,  but  in  general  it  may  be  described  as 
unilateral  and  paroxysmal.  It  is  characteristic  of  most  neuralgias 
that  they  are  not  primarily  localized  in  the  periphery.  The  pain 
seems  to  begin  beneath  the  surface,  and  may  then  shoot  out  to  the 
periphery.  It  may  be  described  as  biting,  boring,  tearing,  darting, 
cutting,  like  an  electrical  shock,  like  a  hot  iron,  etc.,  each  patient 
having  his  own  pet  expression.  It  may  come  and  go  in  lightning-like 
flashes,  or  throbbing  pulsations,  persisting  for  a  shorter  or  longer 
time,  then  stopping  for  minutes,  hours,  or  days,  then  recurring.  When 
continuous,  the  pain  varies  considerably  in  its  intensity. 

The  painful  area  usually  conforms  to  the  peripheral  distribution  of 
the  sensory  nerves.  In  the  herpetic  and  referred  neuralgias  the  root 
zone  area  is  involved. 

Certain  points  seem  to  be  foci  from  which  the  pains  start.  These 
are  usually  situated  along  the  nerve  trunks,  and  pressure  upon  them  is 
often  sufficient  to  cause  an  exacerbation  of  a  mild  attack,  or  to  provoke 
an  attack  in  a  period  of  intermission.  Valleix  attached  considerable 
importance  to  these  points.  They  are  found,  according  to  him: 
(1)  at  the  point  of  emergence  of  the  nerve  trunks;  (2)  at  such  situations 
where  a  nerve  trunk  transverses  a  muscle  to  reach  the  skin;  (3)  at 
points  where  the  nerve  fiber  breaks  up  into  branches;  (4)  at  points 
where  the  nerve  becomes  very  superficial;  (5)  at  Trousseau's 
apophyseal  points.  Valleix's  points  are  of  diagnostic  importance 
particularly  in  separating  the  neuritic  from  the  neuralgic  types. 

Accompanying  phenomena  are  frequent.     In  some  patients  a  sense 


248  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


/■^ 


G 


DK 


Ll- 


Pi)9 


m>l 


\K 


-i5 


Fig.  124. — Cutaneous  reflex  zones  of  hyperalgesia,  showing  their  relations  with  the 
spinal  root  segments  and  their  vegetative  nervous  system  connections.  The  dotted 
areas  are  to  be  referred  to  the  internal  surfaces.     (After  Dejerine.) 


NEURALGIAS 


249 


of  apprehension  may  precede  the  coming  on  of  an  attack;  vague 
sensations  of  discomfort  often  antedate  the  neuralgic  outbreak. 
Ripples  of  pain,  Hke  pin-pricks,  short  twinges,  etc.,  announce  the 
advent  of  a  more  serious  attack,  or  may  be  the  sole  evidence  of  an 
abortive  one.  Such  mild  phenomena  are  extremely  frequent  in 
certain  of  the  so-called  predisposed  or  neuralgic  individuals,  and  they 
feel  that  they  cannot  live  at  high  altitudes;  others  fear  rain,  or  an  east 
wind;  a  thunder  storm  causes  others  to  have  twinges;  while,  again. 


Temporal  CD 7) 


Vertical  W8) 


Orbital  CDS,  3 

Nasofrontal  (.0  3,h) 
Temporofrontal  {D5,6) 


Maxillary 
Nasolabial 

Mental 


'arietal  {DO) 

Occipital  {DIO) 

-Mandibular 
yoicl 


Superior  Laryngeal 
Inferior  Laryngeal 


Fig.  125. — Cutaneous  reflex  zones  of  hyperalgesia  of  the  head,  neck,  and  shoulders  in 
their  relations  to  vegetative  nerve  (somatic)  disturbances.     (After  Dejerine.) 

certain  dietary  indiscretions  make  others  complain  of  painful  twinges 
for  days.  Just  what  conditions  are  at  the  basis  of  these  features 
may  be  difficult  to  run  down.     They  are  none  the  less  real. 

Skin  hypersensitiveness  is  frequent.  It  may  precede  or  accompany 
an  attack,  and  persist  after  the  pain  has  ceased.  Epicritic  sensibilitj^ 
is  mostly  implicated.  Light  touch,  a  pin-prick,  or  slight  degrees  of 
heat  or  cold  are  magnified.  Deep  pressure  and  extremes  of  heat  and 
cold  are  usually  palliative. 


250  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

Anesthesia  is  not  infrequent  following  an  attack  of  pain,  and  the 
exact  topographical  distribution  of  ithe  sensory  modifications  on  the 
skin  throw  considerable  light  on  the  possible  causation  of  a  neuralgia 
(Head). 

Paresthesia  is  very  frequent,  and  certain  distributions  seem  to  show 
it  more  than  others.  Thus,  in  the  cutaneous  branches  of  the  femoral, 
they  are  not  infrequent.  Here  they  take  on  the  character  of  a 
"meralgia  paresthetica." 

Motor  disturbances,  either  as  cramp-like  contractions  or  as  paralyses, 
are  not  infrequent  in  accompanying  conditions.  The  painful  contrac- 
tions of  tic  douloureux  and  the  oculomotor  paresis  of  ophthalmoplegic 
migraine  are  familiar  examples  of  this. 

Vasomotor  and  secretory  symptoms  are  frequent.  The  blood- 
vessels are  frequently  contracted  in  the  early  stages  of  a  neuralgic 
attack,  with  resulting  blanching  and  cooling  of  the  skin.  Following 
this  a  period  of  warmth,  of  redness,  of  free  perspiration  may  result 
from  the  secondary  dilatation  of  the  vessels.  In  many  cases  of  trigem- 
inal neuralgia  other  secretions  may  be  modified.  Crying,  coryza,  or 
salivation  are  not  infrequent,  while  in  widespread  neuralgic  attacks  an 
increase  in  the  amount  of  urine  and  of  milk  secreted  is  frequently  found. 

Premature  graying  of  the  hair,  loss  of  hair,  thickening  of  the  skin, 
erythemata,  eczema,  pemphigus,  herpes,  thickening  of  the  bones, 
and,  occasionally,  muscle  atrophy  are  among  the  rarer  trophic 
by-products. 

During  an  attack,  irregularities  of  the  pulse  are  not  unusual;  slowing 
is  the  rule.     The  pupils  are  frequently  dilated. 

The  general  physical  and  psychical  reactions  are  extremely  impor- 
tant. Loss  of  sleep  and  anorexia  cause  the  patient  to  lose  strength 
and  flesh;  and  anxiety,  irritability,  and  petulance  are  almost  inevitable. 
Mental  depression,  sufficient  to  lead  the  patient  to  make  suicidal 
attempts,  is  not  infrequent,  especially  in  severe  cases  of  trigeminal 
and  sciatic  neuralgia.     The  contracting  of  a  drug  habit  is  not  unusual. 

Course. — This  depends  naturally  upon  the  underlying  condition. 
Many  are  acute  and  transitory,  persist  three  or  four  days  and  never 
reappear.  Such  are  the  herpetic  types.  Many  reflex  neuralgias  run 
an  acute  recoverable  course,  but  show  a  marked  tendency  to  recurrence. 
The  neuralgias  which  accompany  the  chronic  cachexias  of  nephritis, 
carcinoma,  brain  or  spinal  cord  disease,  usually  progress  in  a  markedly 
chronic  manner.  In  those  hereditarily  disposed  individuals  the  ten- 
dency to  chronicity  with  longer  and  shorter  periods  is  proverbial. 
Earlier  French  writers  attempted  to  distinguish  benign  and  severe 
forms.  Most  neuralgias  in  which  the  causative  factor  is  undiscoverable 
(the  so-called  idiopathic  or  primary  neuralgias)  run  a  benign  course, 
while  the  neuritic  types  are  less  amenable  to  treatment. 

The  subdivision  of  neuralgic  neuroses,  subacute  neuritic  neuralgia, 
and  chronic  neuritic  neuralgia  offer  a  grouping  referable  to  course 
which  has  only  clinical  convenience  to  warrant  it. 


NEURALGIAS  251 

In  the  first  type  one  finds  the  disorder  more  or  less  limited  to  the 
neuropath.  The  attacks  come  with  appreciable  cause,  or  follow  a 
nervous  shock.  Exposure  to  cold,  or  dietary  indiscretions  are  fre- 
quently claimed  as  causes,  but  are  not.  The  pain  comes  on  with  great 
suddenness  and  usually  goes  without  great  violence;  it  comes  and  goes 
apparently  without  rhyme  or  reason,  and  is  not  accompanied  by  painful 
nerve  trunk  nor  trophic  disturbances.  It  recovers  at  times,  to  recur 
at  intervals  of  a  year  or  years. 

In  the  subacute  neuritic  neuralgic  type,  exposure  to  cold  or  pressure, 
especially  in  arthritic  patients,  determines  an  attack.  The  attack 
develops  gradually;  the  pain,  at  first  mild  and  intermittent,  gets 
worse  and  worse  and  more  continuous.  Finally,  after  a  day  or  more, 
the  paroxysms  become  extreme,  the  intervals  being  marked  by  dull 
pain;  Valleix's  points  are  characteristic  findings. 

When  a  mixed  nerve  is  involved,  muscular  atrophy  or  other  trophic 
signs  appear,  signalizing  the  occurrence  of  a  neuritic  process.  Local 
edema  and  herpes  zoster  are  frequent  accompaniments.  This  type 
usually  commences  to  recover  in  from  two  to  three  weeks,  and  an 
ultimate  recovery  is  to  be  expected.  Recurrences  occur,  however, 
and  a  leap  to  the  third  type  of  chronic  neuritic  neuralgia  is  made. 
This  form  is  frequent  in  the  aged.  The  history  is  usually  that  of 
several  subacute  attacks  with  increasing  tendency  to  chronicity. 
Here  the  trophic  disturbances  in  muscle  and  in  skin  are  more  marked. 
The  paroxysms  run  a  remittent  course. 

Diagnosis. — Enough  has  been  said  to  emphasize  the  need  for  a 
searching  analysis  of  the  causative  factors  of  every  neuralgia.  They 
are  many,  and  presumably  the  most  widespread  diagnostic  error  is 
the  overlooking  of  an  early  tabes  dorsalis  in  young  to  middle-aged 
adults. 

Children  are  not  prone  to  neuralgias — in  the  narrower  sense — and 
a  neuralgic  affection  in  childhood  calls  for  close  scrutiny. 

Since  unilateral  pain,  of  special  localized  type,  occurring  in  irregular 
attacks,  is  almost  the  sole  criterion  of  neuralgia,  it  is  very  frequent  that 
organic  disease  of  a  viscus  will  show  precisely  similar  accompanying 
features.  In  the  majority  of  cases  the  underlying  organic  lesion  may 
be  detected — occasionally  it  remains  difficult  to  locate.  Not  infre- 
quently the  diagnosis  of  a  persistent  neuralgia  may  be  cleared  up  by 
the  finding  of  malarial  organisms  in  the  blood,  or  more  rarely  the 
presence  of  a  marked  eosinophilia  will  call  attention  to  trichina  as  the 
cause  of  an  obstinate  neuralgia;  or  the  eggs  of  an  intestinal  parasite 
in  the  feces  (uncinaria)  may  direct  attention  to  an  anemia  which 
underlies  a  severe  neuralgia.  Syphilitic  neuralgias,  either  toxic  or 
vascular,  are  by  no  means  infrequent. 

The  diagnosis  of  myalgia  from  true  neuralgia  is  not  often  difficult, 
but  occasionally,  especially  in  the  intercostal  and  lumbar  regions, 
the  diagnosis  becomes  uncertain.  These  neuralgic-like  myalgias  are 
usually  isolated  in  their  location,   are  not,  as  a  rule,  accompanied 


252  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

by  acute  exacerbations,  nor  are  the  regions  usually  painful  on  pressure. 
Motion,  on  the  contrary,  usually  aggravates  myalgias. 

Neuritis  of  a  mild  grade  offers  a  specially  difficult  problem.  As 
already  stated,  mild  neuritis  shows  itself  as  a  neuralgia.  The  question 
to  be  solved  concerns  the  likelihood  of  a  more  severe  degree  of  neuritis. 
In  this  case  the  usual  signs  of  neuritis  are  painful,  swollen  nerve 
trunks,  trophic  disturbances,  more  continuous  pain,  Lasegue's  phe- 
nomenon, weak,  flabby  muscle  fibers,  and  electrical  changes.  New 
growths,  pressing  upon  or  involving  the  nerve  trunks,  within  or  without 
the  spinal  canal,  in  the  early  stages  particularly,  begin  as  pure  neuralgic 
syndromes.  Minute  analysis  of  the  sensory  phenomena  will  usually 
clear  up  the  diagnosis  early,  although  at  times  it  may  be  impossible 
in  the  earliest  stages. 

A  neuralgic  affection  may  be  one  of  the  earliest  signs  of  a  multiple 
sclerosis.  Oppenheim  has  found  a  severe  tic  douloureux  to  have  been 
the  earliest  sign  of  this  disorder.  Syringomyelia  may  begin  as  a 
localized  neuralgia.  Minute  hemorrhagic  lesions  of  the  spinal  cord  of 
traumatic  origin  give  rise  to  neuralgias. 

In  the  diagnosis  of  hysterical  neuralgia,  great  caution  should  be 
exercised.  Hysterical  neuralgias  partaking  of  the  nature  of  a  pseudo- 
neuralgia  are  extremely  diffuse,  and  react  very  rapidly  and  markedly 
to  suggestive  infiuences.  Hysterical  neuralgias  are  almost  invariably 
accompanied  by  other  conversion  signs. 

Neurasthenic  pains  need  to  be  differentiated  if  not  almost  entirely 
ruled  out  as  a  common  diagnostic  pitfall.  The  many  mixed  forms  of 
neurasthenic,  hypochondriacal,  and  hysterical  neuralgic  pains  should 
bear  their  characteristic  sidelights.  The  diagnosis  of  these  types  of 
neuralgia  should  not  be  lightly  made,  for  it  is  not  to  be  forgotten  that 
these  syndromes  of  themselves  may  be  the  reaction  on  the  part  of  the 
nervous  system  to  some  more  fundamental  organic  lesion.  Thus 
patients  suffering  from  severe  so-called  neurasthenia  with  cachexia, 
and  severe  intercostal  neuralgic  pains  may  have  an  undiscovered 
carcinoma  of  the  stomach,  mediastinum,  etc. 

In  tabes  dorsalis  the  neuralgic  pains  have  a  wide  range,  are  rarely 
localized  in  a  peripheral  nerve  distribution,  and  are  apt  to  be  radicular 
in  their  distribution.  Pain  on  pressure  of  the  nerve  trunk  is  usually 
absent.  The  objective  findings  in  the  pupils  and  cerebrospinal  fluid 
establishes  the  diagnosis. 

The  pains,  cramps,  and  muscular  weakness  of  intermittent  claudica- 
tion sometimes  gives  rise  to  severe  neuralgias.  Aortic  aneurism  gives 
rise  to  reflex  neuralgic  pains,  which  are  usually  very  severe,  burning  or 
boring  in  character.  Aneurisms  in  other  regions  are  to  be  carefully 
excluded. 

In  reflex  neuralgias  the  use  of  cocain  or  other  local  anesthetic  may 
determine,  by  exclusion,  the  site  of  the  original  lesion.  An  orthoform 
suppository  pressed  well  against  the  prostate  has  been  known  to  relieve 
a  severe  sciatic  neuralgia.     Tumors  of  the  pelvis  frequently  give  rise 


NEURALGIAS  253 

to  sciatic  and  crural  neuralgias,  and  persistent  neuralgic  pains  of  the 
knee  are  often  a  reflex  from  hip-joint  disorder. 

Neuralgic  pains  are  a  frequent  indication  of  disturbed  nerve  fiber 
metabolism,  with  a  hyper-  or  a  hypothyroid  affection  behind  it. 

For  the  precise  localization  of  the  areas  involved  consult  Figs.  11, 
12,  13,  14,  15  and  16,  where  both  the  peripheral  and  radicular  nerve 
distribution  are  figured. 

Prognosis. — This  is  conditioned  by  the  pathological  process  that  is 
responsible.  The  more  chronic  of  the  neuralgias,  which  in  years  gone 
by  tended  to  bring  about  chronic  invalidism  or  inveterate  drug  habits, 
have  ceased  to  have  such  a  sinister  import  by  means  of  a  better  under- 
standing of  the  underlying  conditions,  and  by  a  much  more  resourceful 
therapy.  The  younger  and  stronger  the  individual,  and  the  less  the 
tendency  to  hereditary  disposition,  the  better  the  prognosis  in  those 
neuralgias  which  apparently  are  idiopathic,  as  well  as  those  due  to 
alcohol,  lead,  or  other  toxic  agent.  In  the  more  chronic  forms 
which  are  not  due  to  removable  condition  the  prognosis  is  bad. 
With  increasing  insight,  however,  into  the  many  intricate  disturb- 
ances of  nerve  metabolism  many  of  the  intractable  forms  may  be 
conquered. 

Treatment. — The  chief  indications  are  to  quiet  the  pain  and  ascer- 
tain the  cause.  A  painstaking  study  of  the  history,  and  an  exhaustive 
physical  examination,  are  necessary  in  all  cases.  The  therapy  will 
vary,  therefore,  widely  if  the  cause  be  ascertained;  quinin  will  cure 
one  patient,  a  surgical  operation  may  be  called  for  in  another.  General 
rules,  therefore,  are  largely  illusory.  One  should  never  treat  a  neu- 
ralgia per  se,  as  it  is  solely  a  general  result  of  many  causes. 

Taking  up  the  general  therapeutic  indications,  the  analgesics  which 
have  proved  useful  may  be  discussed  first.  Phenacetin,  acetanilid, 
antipyrin,  aspirin,  pyramidon,  lactophenin,  and  phenocoll  are  among 
those  that  have  been  valuable.  New  ones  are  constantly  being  added, 
and  among  them  some  are  certain  to  be  of  value.  The  salicylic  acid 
group  combinations  are  at  times  useful,  especially  in  the  milder  cases 
and  in  patients  with  arthritic  tendencies.  In  influenza  and  tonsillitis 
neuralgias  the  salicylates  are  useful.  Combinations  of  these  with 
soporifics,  such  as  chloral,  paraldehyde,  sulphonal,  trional,  or  veronal, 
are  useful  in  procuring  sleep,  and  thus  prevent  the  reduction  of  the 
patient's  resistance. 

If  any  of  the  opium  group  be  necessary  it  is  better  to  give  such  in 
sufficient  doses.  Usually  smaller  doses  may  be  given  when  combined 
with  the  analgesics  mentioned.  Aspirin,  gr.  vij  (0.5  gram),  codein, 
gr.  I  (0.02  gram),  and  trional,  gr.  vij  (0.5  gram),  for  instance,  is  a  useful 
combination  to  be  taken  at  night.  Other  combinations  are  equally 
effective.  In  the  chronic  neuralgic  pains  morphin  is  to  be  avoided  as 
long  as  possible.  This  does  not  apply  to  a  very  old  patient,  or  one  in 
whom  the  neuralgia  is  simply  the  expression  of  some  chronic  incurable 
disorder,  carcinoma  for  example.     The  gradually  acquired  immunity, 


254  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

with  the  need  for  larger  doses,  and  the  pernicious  eflfeets  of  a  habit 
apply  to  all  the  members  of  the  opium  group. 

All  analgesics  are  purely  provisionally  used.  They  are  meant  to 
give  the  patient  ease  while  looking  for  the  real  cause  of  the  pains. 

If  one's  inquiry  is  satisfied  by  the  relief  of  pain  the  use  of  analgesics 
alone  is  bad  therapy.  Other  drugs  are  quinin,  which  in  combination 
with  the  salicylates  is  specially  valuable ;  arsenic  which  is  serviceable  in 
the  neuralgias  due  to  anemia,  especially  in  combination  with  iron. 
Atropin  and  aconitin  were  used  widely  before  the  days  of  the  antipyretic 
analgesics.  Their  definitely  poisonous  qualities  have  driven  them  into 
the  background.  The  unreliability  of  cannabis  indica  has  done  the 
same  for  this  otherwise  useful  analgesic.  The  iodides  are  called  for 
in  the  syphilitic  neuralgias  and  are  useful  in  many  neuritic  neuralgias. 

Counter-irritation  is  of  great  service  in  many  cases  of  severe  neu- 
ralgia, especially  after  the  acute  onset  is  over.  The  Paquelin  cautery 
is  the  best  means ;  mustard  paste,  cantharides,  turpentine,  chloroform, 
ether,  and  acupuncture  all  have  their  place.  Local  freezing  may  be 
carried  out  by  ethyl  chloride,  methyl  chloride,  ether,  or  other  volatile 
substances.  Menthol,  or  other  similar  derivatives  may  be  used  for 
mild  neuralgic  pains  to  advantage. 

Direct  applications  of  local  analgesics,  either  to  the  nerve  trunk  or 
within  the  spinal  canal,  are  valuable  in  many  deep-seated  neuralgias, 
especially  of  medullary  origin.  Cocain,  tropococain,  eucain  are  all 
useful  given  by  the  Corning  or  Quincke  method. 

Local  applications  of  heat  are  grateful  and  valuable.  Hot  water 
bags,  hot  sand,  electrical  pads,  etc.,  may  be  utilized.  General  or  local 
hot-water  baths  or  hot-air  baths  (baking)  are  at  times  desirable. 

General  hygienic  treatment  is  imperative.  A  generous  diet,  full 
sleep,  healthful  occupation,  and  freedom  from  mental  worry  are 
essential.  Cod-liver  oil,  nitrogenous  diet,  with  iron,  arsenic,  strychnin, 
calcium  salts,  are  indicated.  Faddy  dietaries  should  be  avoided. 
Even  in  arthritic  neuralgias  it  is  doubtful  if  meat  does  any  particular 
harm  when  not  taken  to  excess.     Alcoholic  beverages  are  to  be  denied. 

An  alkaline  therapy  often  helps  many  fugacious,  persistent  neuralgic 
pains.     Fruits  containing  the  citrates  seem  to  give  relief. 

Preparations  of  the  internal  secretions,  particularly  thyroid  and 
pituitary  clear  up  some  intractable  neuralgias  of  unknown  origin. 
They  may  be  given  in  doses  of  from  yV  to  y  grams  twice  or  thrice  daily. 

Climatic  changes  are  rarely  advisable.  Low-lying,  damp  and  humid 
atmospheric  conditions  seem  least  desirable  for  certain  patients.  The 
general  stimulus  that  comes  from  a  dryer,  higher  atmosphere,  even  if 
colder,  works  to  the  general  advantage,  even  if  not  directly  valuable 
for  the  relief  of  pain. 

Electrotherapy  when  well  managed  and  properly  selected  is  of  great 
value  in  some  neuralgias.  It  cannot  be  said  that  it  is  clearly  recognized 
just  what  forms  of  current  are  best  utilizable  in  what  types  of  neu- 
ralgia, hence  most  efforts  must  follow  the  method  of  trial  and  error. 


TRIGEMINAL  NEURALGIA  255 

In  general,  however,  Leduc's  modifications  of  d'Arsonval's  rapidly 
interrupted  current  offer  the  readiest  and  most  widely  applicable  form 
of  electrical  current  for  the  relief  of  neuralgic  pain.  It  is  doubtful  if 
any  other  form  of  electrical  application  is  known  at  the  present  time 
that  is  as  valuable  as  this.  It  is,  in  fact,  a  type  of  electrical  anesthesia, 
solely  palliative,  but  very  grateful.  Newer  applications  are  being 
brought  out,  and  other  forms  may  replace  the  Leduc  currents,  but 
at  present  these  seem  to  give  the  most  reliable  results. 

Faradic  currents,  as  heretofore  employed,  act  for  the  most  part 
simply  as  counter-irritants,  and  seem  to  possess  little  superiority  over 
the  actual  cautery.  Galvanism  with  mild  currents  is  useful  for  many 
topalgias. 

Psychotherapy  is  the  only  rational  treatment  for  the  psychogenic 
neuralgias.    They  make  up  nearly  25  per  cent,  of  the  neuralgias. 

Surgical  intervention  is  called  for  in  all  cases  in  which  pressure  is 
demonstrable  and  the  cause  removable.  Tumors  and  new  growths, 
involving  or  pressing  upon  nerve  structures,  if  removable  should  be 
taken  away.  Surgical  interference  may  be  of  radical  service  in  many 
of  the  reflex  neuralgias  of  obscure  origin,  probably  related  to  visceral 
ptoses.  Such  surgical  interference  is  justifiable  only  in  chronic  cases 
where  these  visceral  ptoses  have  resulted  from  long-continued  psychical 
causes.  Psychotherapy  is  preferable  in  the  early  stages.  Nerve 
stretching  needs  mention  mostly  to  be  condemned. 


SPECIAL  LOCALIZED  FORMS  OF  NEURALGIAS. 

While  any  sensory  nerve  in  the  body  may  become  painful,  there 
are  certain  regions  which  show  a  greater  tendency  to  involvement 
than  others.  Bernhardt  has  collected  the  statistics  of  localized 
distribution  in  some  685  cases,  with  the  following  results:  trigeminal, 
124;  occipital,  42;  brachial,  108;  intercostal,  45;  lumbo-abdominal, 
12;  crural,  25;  obturator,  2;  sciatic,  303;  anterior  femoral,  11;  Achilles, 
3;  tarsalgia,  4;  metatarsalgia,  4;  and  coccygeal,  2.  In  616  cases 
collected  by  one  of  us  (J.)  during  four  years  (1902  to  1906)  the  dis- 
tribution was  as  follows:  trigeminal  315,  occipital,  28;  brachial,  31; 
ulnar,  1;  intercostal,  19;  lumbo-abdominal,  19;  crural,  2;  sciatic,  194; 
coccygeal,  1 ;  peroneal,  2 ;  and  plantar  1 . 

Trigeminal  Neuralgia. — Simple  neuralgia  of  the  branches  of  the 
fifth  nerve  are  among  the  commonest  of  all  the  neuralgias.  Fother- 
gill's  studies  on  A  Painful  Affection  of  the  Face,  published  in  1773,  is  a 
classic.  The  inferior  and  superior  branches  preponderate  in  frequency 
of  involvement.  Most  frequently  these  neuralgic  pains  are  due  to 
some  affection  of  one  of  the  branches.  Inflamed  teeth  play  a  pre- 
dominant role.  Affections  of  the  ears,  the  eyes,  iritis,  cyclitis,  iridocy- 
clitis, the  skin  of  the  face  or  head,  inflammation  within  the  accessory 
sinuses  of  the  nose,  forehead,  antrum,  mastoid,  all  of  these  may  produce 


256  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

diffuse  neuralgic  pains,  at  times  clearly  separable  from  a  neuritic 
neuralgia  of  the  fifth,  at  other  times  not. 

Cold  and  wet  are  important  agents  in  facial  neuralgia.  In  certain 
countries,  notably  England  and  the  north  of  Germany,  trigeminal 
neuralgias  from  this  cause  are  extremely  common;  they  seem  to  be 
much  less  frequent  in  the  United  States,  and  notably  so  in  southern 
countries. 

Neuralgia  of  the  superior  branch  is  seen  more  commonly  by 
physicians  although  the  dental  branches  are  involved  much  more 
frequently.  These  patients  go  to  dentists  and  therefore  do  not  enter 
into  medical  statistics.  This  is  a  reason  why  it  is  incorrectly  stated 
by  most  writers,  that  the  superior  branches  of  the  fifth  are  most  often 
involved.  For  the  most  part  the  milder  types  of  neuralgia  are  induced 
by  irritation  of  some  of  the  terminal  filaments,  while  in  the  neuritic 
form,  tic  douloureux,  which  is  the  more  classic,  a  lesion  of  the  Gasserian 
ganglion  is  usually  present.  Mild  cases  of  tic  douloureux  may  be  indis- 
tinguishable clinically  from  other  types  of  neuralgic  pain. 

Tic  Douloureux. — Enough  has  been  said  on  neuralgia  in  general  to 
indicate  the  character  of  the  simpler  form  of  neuralgia  of  the  fifth. 
One  type,  however,  by  reason  of  its  severity  and  its  fairly  definite 
pathological  anatomy,  needs  more  extended  consideration.  Avicenna 
knew  tic  douloureux  and  described  it  with  great  accuracy.  It  would 
be  desirable  to  restrict  the  term  tic  douloureux  to  a  definite  and,  if 
possible,  limited  type  of  neuritis  of  the  fifth  nerve,  particularly  to  the 
form  due  to  changes  of  a  chronic  degenerative  nature  occurring  in  the 
Gasserian  ganglion.  This  is  not  yet  possible,  and  clinically  the  neuritic, 
and  peripheral  neuralgic  cases  are  either  not  at  all  separable  from  the 
ganglion  cases,  or  with  considerable  difficulty. 

Tic  douloureux  usually  affects  one  side  of  the  face.  In  the  majority 
of  cases  some  selection  occurs  among  the  branches,  one  or  two  being 
involved,  rarely  all  three;  the  ophthalmic  branch  the  oftenest,  the 
inferior  maxillary  the  least  often. 

The  more  classical  tic  douloureux  neuralgias  are  characterized  by 
the  extreme  severity  of  the  pain,  usually  preceded  by  paresthetic 
prodromata,  and  widely  accompanied  by  sympathetic  or  irradiating 
pains  in  other  branches  than  the  one  chiefly  involved,  or  in  other  nerves. 
The  pain  may  be  paroxysmal  or  continuous,  with  marked  exacerbations. 
Patients  compare  them  to  the  piercing  pains  of  a  sharp  knife  or  the 
burning  of  a  red-hot  wire.  The  patient  remains  for  a  shorter  or  longer 
period,  a  few  minutes  to  several  hours,  under  the  grip  of  the  pain, 
unable  to  move  a  muscle  of  the  face  or  fearful  of  stirring,  lest  a  spasm 
more  fearful  than  the  others  should  occur;  even  the  air  pressure  of  a 
suddenly  closed  door  may  bring  on  an  exacerbation.  The  longer 
attacks  are  rarely  as  vicious  as  the  shorter  ones. 

Valleix's  points  are  relatively  constant.  In  ophthalmic  involve- 
ment the  sore  points  are  found  above  the  supraorbital  notch,  at  the 
external  angle  of  the  upper  lid,  the  upper  outer  aspect  of  the  nose,  and 


TIC  DOULOUREUX  257 

the  globe  of  the  eye;  in  the  superior  maxillary  branch  the  inferior 
orbital  notch  is  the  chief  point  of  pain;  the  malar  bone,  and  opposite 
the  last  upper  molar  are  other  less  frequently  found  points,  while  the 
outer  angle  of  the  mouth,  and  the  roof  of  the  mouth  are  rarely  their 
site.  In  the  inferior  maxillary  distribution  the  points  are  chiefly  just 
in  front  of  the  auditory  canal,  the  side  of  the  tongue,  the  border  of  the 
chin,  and  Trousseau's  points  over  the  first  and  second  cervical  vertebrae. 

Vasomotor  and  secretory  disturbances  are  usual.  The  skin  is,  as  a 
rule,  hot  and  swollen,  occasionally  pale  and  frigid;  tears,  nasal  secre- 
tions, and'  saliva  flow  in  abundance.  The  eyelids  may  be  swollen, 
the  conjunctiva  reddened  to  the  point  of  ulceration  at  times;  within 
the  nose  and  mouth  extravasations  occur,  and  ulcers  are  not  uncommon. 
Herpetic  attacks  are  also  not  infrequent,  and  in  some  of  these  attacks 
grave  injury  to  the  eye  structures  may  take  place.  Glaucoma  is  one 
of  the  severe  complications.  Other  trophic  disturbances  are  skin 
eruptions,  acne,  erysipelatous  reddening,  graying  of  hair,  and  blacken- 
ing of  the  tongue.  In  long-continued  cases  hemiatrophy  may  occur. 
Changes  in  the  sense  of  taste,  of  touch,  of  hearing,  are  at  times  present. 
Photophobia  is  frequent,  while  diminution  in  the  visual  fields  and 
accommodation  cramps  are  noted. 

Severe  mental  disturbance,  amounting  at  times  to  hallucinatory 
confusion  may  be  present.  Suicidal  attempts  are  to  be  guarded  against 
in  these  excruciating  cases. 

The  motor  disturbances  consist  in  convulsive  movements  of  the 
facial  muscles  (convulsive  tics,  spasmodic  epileptiform  neuralgia, 
Trousseau),  sudden  forced  closing  of  the  eyelids,  drawing  of  the  mouth 
to  one  side,  or  sudden  turning  of  the  head.  At  times  the  convulsive 
movements  extend  to  the  arms.  Paralytic  phenomena  in  the  third 
nerve  are  noted.  The  general  psychical  disturbances  noted  are  prone 
to  occur  in  this  type. 

Course.- — In  the  majority  of  cases  the  attacks  appear  in  series  and 
attain  a  periodicity  which  comes  to  be  dreaded  by  the  sufferer.  The 
free  intervals  usually  become  shorter  and  shorter;  but  many  patients 
may  have  only  one  attack  a  year,  especially  in  cold  weather,  or  even 
at  longer  intervals.  A  single  attack  may  last  a  few  days,  or  in  the 
severe  forms  several  weeks,  the  patient  not  being  free  from  pain  day 
or  night,  save  under  the  influence  of  morphin.  Some  patients  have  a 
few  attacks  in  a  lifetime,  others  are  not  free  from  the  disease  for  years. 
The  severer  convulsive  forms  are  prone  to  occur  late  in  life. 

Diagnosis. — Ordinarily  the  classical  form  of  tic  douloureux  is 
recognized  without  difficulty.  Patients  have  all  their  teeth  extracted, 
however,  under  the  mistaken  diagnosis  of  a  dental  disease,  while 
some  intractable  trigeminal  neuralgias  have  been  cured  by  proper 
attention  to  diseased  teeth.  Aneurism  of  the  carotid,  tumors  pressing 
upon  the  nerve  or  upon  the  Gasserian  ganglion,  may  be  diflScult  to 
determine  as  the  exciting  cause.  These,  however,  are  usually  accom- 
panied by  accessory  symptoms,  palsies,  eye-ground  changes,  aneuris- 
17 


258  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

mal  murmurs,  pain  within  the  head,  cerebellar  syndromes,  ear  pains, 
etc.  The  otalgias  (tympanic  neuralgias)  usually  considered  in  this 
connection  are  possibly  due  to  geniculate  ganglion  disorder,  and  have 
been  referred  to  by  Hunt  as  neuralgias  of  the  seventh  nerve. 

Multiple  sclerosis  has  started  as  a  trigeminal  neuralgia. 

Treatment. — It  is  as  essential  to  endeavor  to  find  and  treat  the  cause 
for  a  facial  neuralgia  as  for  neuralgia  in  general.  The  various  remedies 
given  under  the  heading  of  neuralgia  may  be  tried,  and  as  malarial 
neuralgias  are  very  frequently  trigeminal,  energetic  quinin  therapy 
should  be  given;  the  absence  of  blood  findings  is  not  contra- indicative, 
especially  in  non-malarial  neuralgias.  Gelsemium,  the  tincture  in  10 
minim  doses,  gradually  ascending,  aconite  in  doses  of  g-g-Q  grain, 
cannabis  indica  (fresh),  in  doses  of  from  I  to  ^  grain,  is  reputed  as 
specially  valuable  in  the  facial  cases.  Any  of  the  analgesic  antipyretics 
may  suit  individual  cases,  and  avoid  the  use  of  morphin,  which  alone 
is  reliable  in  many  severe  cases.  Local  applications  of  cocaine  to  the 
conjunctiva,  nasal  mucous  membranes,  buccal  surfaces  are  sufficient 
to  repress  some  mild  attacks. 

Injection  methods  have  been  tried  for  years.  In  the  beginning  the 
peripheral  branches  were  injected  by  various  analgesic  drugs,  in  early 
days  chloroform,  and  in  later  times  particularly  cocaine  and  its  allies 
or  derivatives.  The  effects  were  valuable,  but  temporary.  Osmic 
acid  was  used  later,  but  regeneration  took  place.  Pitres  and  Vaillard, 
in  1887,  and  Schlosser,^  in  1900,  took  up  a  series  of  experiments  with 
alcohol,  and  the  latter  has  perfected  a  method  of  injecting  alcohol 
within  the  substance  of  the  Gasserian  ganglion,  which  has  given  excel- 
lent results.  The  chief  features  in  the  deep  alcohol  injection  method 
is  the  introduction  of  a  long,  dull,  cutting  needle  into  the  foramen 
ovale  and  there  injecting  in  situ  the  branches  of  the  trigeminus. 
Special  methods  have  been  devised.  Narcosis  is  not  necessary;  80 
per  cent,  alcohol  is  used.  In  three  to  four  hours  following  the  injec- 
tion the  pain  is  relieved,  and  two  or  three  more  injections  are  given 
within  a  week  to  complete  the  treatment.  Immediately  following 
the  injections,  which  should  be  done  only  after  extended  practice  on 
the  cadaver,  there  is  a  marked  anesthesia  on  one  side  of  the  anterior 
part  of  the  head,  including  the  nostril,  palate,  and  one-half  of  the 
tongue;  a  slight  paralysis  of  the  muscles  of  mastication,  which  may 
persist  for  some  time,  but  usually  disappears  in  a  few  hours;  a  degen- 
erative process  is  set  up  in  the  nerve  trunk,  which  is  recoverable,  and 
general  sensibility  usually  returns,  but  the  pain  returns.  Relief  extend- 
ing over  a  year  in  a  number  of  cases  is  reported  by  numerous  observers. 
Some  patients  have  been  relieved  for  four  or  five  years.  Edema  of  the 
posterior  eye  structures  and  hemorrhage  are  among  the  discomforts 
and  even  dangers  of  the  operation,  especially  in  the  use  of  the  intra-or- 
bital  methods  devised  by  French  operators.    Relapses  are  apt  to  occur. 

1  Munch,  med.  Woch.,  April  30,  1897. 


CERVICO-OCCIPITAL  NEURALGIA  259 

Three  surgical  procedures  have  been  seriously  advocated.  The  first 
and  earliest  consisted  of  peripheral  section,  first  said  to  have  been 
done  by  J.  C.  Warren  of  Boston.  Section  of  the  fifth  may  be  employed 
to  advantage  in  those  cases  in  which  the  disease  is  undoubtedly  peri- 
pheral. As  modified  by  more  recent  procedures,  the  older  objection 
that  regeneration  takes  place  is  partly  done  away  with. 

Rose,  MacEwen,  Horsley,  Hartley,  and  Krause  perfected  the  opera- 
tion of  excision  of  the  ganglion,  and  the  modified  Hartley-Krause 
operation  by  the  temporal  route  has  been  largely  the  method  of 
choice.  Cushing's  more  recent  modifications  are  of  lasting  value. 
The  operation  still  remains  one  of  much  difficulty  and  seriousness. 
Recurrences  are  known  even  with  this  method,  and  the  efficiency  of 
the  newer  devices  for  preventing  this  by  capping  the  ends  of  the 
divided  nerve  trunks  with  metallic  laminae  is  too  recent  to  pronounce 
upon. 

Van  Gehuchten,  in  1903,  suggested  tearing.  Spiller,  in  1898,  had 
suggested  the  surgical  expedient  of  cutting  the  sensory  root,  which 
he  claims  is  safer  than  the  operation  of  tearing,  a  procedure  tried  in 
1881.  The  method  of  division  of  the  sensory  root,  as  reported  by 
Frazer  and  Spiller,  promises  to  be  one  of  the  most  valuable  surgical 
procedures  thus  far  devised. 

Cervico-occipital  Neuralgia. — This  occurs  in  the  distribution  of  the 
sensory  nerves  of  the  cervical  plexus,  consisting  chiefly  of  the  occip- 
italis major,  the  occipitalis  minor,  auricularis  magnus,  cervicalis 
superior,  supraclavicularis  and  phrenic.  Neuralgia  in  this  general 
region  seems  to  be  rare.  In  Remak's  summary  of  15,000  cases  only  50 
were  in  the  cervico-occipital  regions.  Valleix  has  given  one  of  the  most 
complete  monographs  on  neuralgia  in  this  area  and  little  has  been 
added  to  his  description,  save  in  the  finding  of  rare  etiological 
factors. 

Etiology. — The  several  causes  of  neuralgia  are  operative  here  and 
need  not  be  repeated.  Special  determining  features  seem  to  be  the 
carrying  of  heavy  weights  on  the  shoulders  (a  more  frequent  cause 
seen  in  brachial  neuralgias),  arthritis  deformans  of  the  upper  cervical 
vertebrae,  caries,  syphilis,  tuberculosis,  tumors,  cervical  pachymenin- 
gitis, falls  and  blows  wrenching  the  cervical  vertebrae,  enlargement 
of  the  cervical  lymphatics,  and  aneurisms  of  the  vertebral  artery. 
Oppenheim  refers  to  the  great  frequency  of  hysterical  neuralgia  in 
this  region  and  psychogenic  neuralgias  of  the  back  of  the  neck  and 
occiput  are  extremely  common.  They  are  often  found  in  individuals 
who  are  either  under  great  strain  or  those  who  are  constantly  forcing 
themselves. 

The  pains  occupy  the  regions  mentioned,  being  particularly  local- 
ized in  the  neck,  below  the  occiput,  and  running  up  to  the  vertex, 
occasionally  behind  the  ears.  The  Valleix  point  found  most  frequently 
is  the  occipital  point  between  the  mastoid  apophysis  and  the  first 
cervical  vertebra;    points  between  the  sternomastoid  and  trapezius 


260  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

(cervical),  the  anterior  border  of  the  mastoid,  and  the  middle  of  the 
ear  are  of  less  frequent  occurrence. 

The  pain  is  frequently  bilateral.  Dull  pain  on  pressure,  with  tender 
skin,  is  usual  as  a  paroxysmal  occurrence.  This  tends  to  make  the 
sufferer  hold  his  head  in  a  stiff  position,  which  in  time  may  cause  a 
characteristic  attitude.  This  tenderness  may  be  so  acute  that  ruffling 
of  the  hair  will  start  a  paroxysm.  Graying  of  the  hair,  loss  of  hair, 
with  other  trophic  signs  may  be  present.  Sudden  pulling  back  of  the 
head,  or  other  muscular  involvement,  is  an  occasional  symptom. 

Diaphragmatic  Neuralgia. — ^This  form  of  neuralgia,  also  known  as 
phrenic  neuralgia,  is  of  rare  occurrence.  Falot  and  Peter  have  written 
upon  it.  The  pain  is  usually  present  near  the  free  border  of  the  ribs, 
occasionally  as  high  as  the  chin  and  in  the  neck,  beneath  the  clavicle, 
and  in  the  scalenus  anticus  muscle.  Trousseau's  points  are  located 
over  the  second  to  the  fifth  cervical  vertebra.  The  pain  frequently 
runs  down  the  arm,  especially  in  certain  complex  cases  of  mixed 
brachial  neuralgia. 

Breathing  may  be  seriously  interfered  with,  the  breath  coming  fast 
and  short;  longer  excursions  of  the  diaphragm  are  impossible.  It 
is  a  common  experience  to  have  a  short,  sharp  stitch  in  the  side,  with 
inability  to  breathe  for  fear  of  pain.  This  is  the  type  of  distress  en- 
countered in  phrenic  neuralgia.  In  the  majority  of  cases  the  pain  is 
in  the  left  side. 

Anemia,  affections  of  the  mediastinum,  heart  and  pericardium,  and 
aneurism  of  the  aorta  are  the  most  frequent  attending  features.  An 
intractable  phrenic  neuralgia  may  comphcate  an  exophthalmic  goitre, 
or  be  present  in  carcinoma  of  the  neck  region. 

Idiopathic  or  pure  phrenic  neuralgias  seem  to  be  unusual,  whereas 
temporary  or  more  permanent  types  are  seen  as  symptoms  of  the 
affections  named.  In  the  latter  case  the  prognosis  depends  on  the 
initial  difficulty. 

Brachial  Neuralgia. — In  this  general  form  the  components  of  the 
brachial  plexus,  from  the  four  lower  cervical,  or  some  of  its  filaments, 
and  first  dorsal  roots,  are  those  involved.  The  chief  nerves  carrying 
sensations  from  the  skin  area  of  the  arms  and  shoulders  are  the  cir- 
cumflex, radial,  internal  cutaneous,  and  musculocutaneous.  These 
enter,  for  the  most  part,  the  upper  and  middle  cords  of  the  plexus. 
In  the  majority  of  cases  the  pains  of  brachial  neuralgia  are  located  in 
the  upper  arm  and  about  the  shoulder,  i.  e.,  in  the  area  of  the  circumflex, 
radial,  musculocutaneous,  and  internal  cutaneous  nerves. 

Bernhardt's  statistics  show  that  men  are  more  frequently  affected 
than  women,  but  the  reverse  shows  true  in  the  figures  of  other  observers 
(Romberg,  Erb).  More  women  have  brachial  neuralgia  than  men,  and 
in  most  instances  it  seems  that  excessive  sweeping  is  the  attributed 
cause.  In  piano-players,  neuralgias  in  this  area  are  frequent.  Perhaps 
these  should  be  relegated  to  the  occupation  neuroses  with  the  pains 
of  hair-dressing,  skirt-carrying,  telegraphy,  writing,  etc.    At  any  rate, 


BRACHIAL  NEURALGIA 


2G1 


arm  and  shoulder  pains  are  frequent,  in  their  mild  grades  at  least, 
and  very  variable.  (See  Fig.  126  and  Figs.  11,  12,  13,  14,  15,  and  16.) 
The  usual  causative  factors  come  into  play  here.  The  neuropathic 
constitution  is  put  in  the  foreground  by  Oppenheim;  Bernhardt  lays 
considerable  stress  upon  the  importance  of  bone  injuries  with  callus 
formation  in  the  causation  of  many  arm  neuralgias.  Small  punctured 
wounds  about  the  forearm,  wrist,  and  arm  are  responsible  for  many 
symptomatic  neuralgias,  as  Weir  Mitchell  has  so  well  shown.  More 
remote  cases  are  found  in  vertebral  disease,  tumor  formation,  aneu- 
risms, syringomyelia,  multiple  sclerosis,  and  tabes.  The  frank  neuritic 
processes  in  their  beginnings  must  be  borne  in  mind,  and  cervical 
rib  should  not  be  overlooked. 


FIFTH 
CERVICAU 


SIXTH 


SEVENTH 


TO  SCALENI  & 
LONGUS  COLLI 


THI^RD 
POSTERIOR 


-FROM  FOURTH  CERVICAL 


SUPRASCAPULAR 

XTERNAL  ANTERIOR  THORACIC 

NTERNAL  ANTERIOR  THORACIC 


C/y CIRCUMFLEX 


■S MUSCULO  SPIRAL 


Fig.  126. — Plan  of  the  brachial  plexus.     (Gerrish.) 


Symptoms. — Cervicobrachial  neuralgias  are  extremely  variable  in 
distribution,  extent,  and  severity.  The  onset  is  usually  sudden,  espe- 
cially in  those  patients  in  whom  an  antecedent  history  of  exposure  to 
cold  and  to  wet  is  obtainable  (motormen,  policemen,  etc.);  at  times 
the  beginning  is  preceded  by  twinges  and  slight  distress.  On  awakening 
in  the  morning  sharp  pain  is  felt  in  the  shoulder  and  arm.  The  pains 
in  brachial  neuralgia  are  less  apt  to  be  the  sharp,  shooting  variety  so 
dreaded  in  tic  douloureux,  but  sudden  accessions  of  sharp  pains,  vary- 
ing in  their  intensity,  are  frequent.  As  with  most  neuralgic  pains, 
movement  increases  them.    Toward  evening  the  pains  are  apt  to  in- 


262 


AFFECTIONS  OF   THE  PERIPHERAL  NERVES 


crease  and  the  patient,  although  obtaining  relief  by  lying  down,  rarely 
sleeps  well.  Soreness  of  the  skin,  slight  swelling,  and  general  reduction 
in  tone  are  the  usual  accompaniments.  With  increasing  disuse  slight 
atrophy  is  common,  and  swelling  is  usual.  The  tendon  reflexes  are 
usually  more  irritable  and  active.  More  atrophy  paresis  with  vaso- 
motor-trophic  symptoms  and  altered  tendon  reflexes  indicate  a  definite 
neuritic  process.  Herpetic  eruptions  occur  with  non-infectious,  as 
well  as  with  infectious  involvement  of  the  sensory  ganglia. 


Fig.  127. — Painful  points  in  brachial  neuralgia. 


Tender  points  are  very  variable.  They  are  most  frequently  in  the 
middle  of  the  back;  about  the  level  of  the  second  or  third  dorsal  there 
is  usually  a  sore  Trousseau  point.  Gowers  notes  that  the  inferior 
ulnar  point  in  front  of  the  wrist  is  the  commonest  sore  point.  Babinski 
has  called  particular  attention  to  a  radial  neuralgia  due  to  a  mild  or 
severe  neuritis  of  the  radial.  The  pains  occupy  the  posterior  portion 
of  the  arm,  and  are  unusually  severe.  Neuritic  changes  are  not  infre- 
quent. The  chief  causes  seem  to  be  exposure  to  cold  and  disturbances 
of  the  reciprocal  action  of  the  ovaries  and  thyroids  or  testicles  and 
other  endocrinous  glands.  The  menopause  is  a  frequent  period  of 
onset. 


INTERCOSTAL  NEURALGIA  263 

Diagnosis. — In  the  diagnosis  particular  care  is  needed  in  excluding 
affections  of  the  spinal  cord,  meninges,  and  vertebrae,  as  well  as  angina 
pectoris  and  pseudo-angina.  Disease  of  the  joints  and  bones  should 
be  excluded  at  the  outset,  although  it  may  be  impossible  in  some 
cases  of  periostitis.  In  tumors  and  other  organic  affections  of  the  cord 
the  painful  points  are  usually  absent,  but  the  earliest  and  only  symptom 
of  spinal  cord  tumor,  intramedullary  or  extramedullary,  may  be  a 
brachial  neuralgia.  In  tabes  the  pains  are  apt  to  be  bilateral.  The 
exhaustion  neuralgias  are  also  apt  to  be  bilateral.  Valleix's  and  Trous- 
seau's points  are  usually  absent.  The  general  indefinite  features  of  a 
myalgia,  plus  the  muscular,  rather  than  the  nerve  soreness,  are  usually 
sufficient  to  exclude  it. 

The  occupation  neuroses  involving  the  arm  and  shoulder  are  many. 
The  history  of  protracted  exercise  of  certain  groups  of  muscles  is 
usually  sufficient  to  identify  the  proper  cause  for  the  neuralgic  pains. 
Occupation  neuralgias,  like  neuritic  neuralgias,  are  neuralgias  none  the 
less,  the  sole  diagnostic  question  arising  as  to  the  cause,  and  through 
this  the  proper  mode  of  therapeutic  attack  and  the  probable  outcome. 
Alcoholic  neuritis  in  its  mild  grade  offers  particular  embarrassments. 
Lead  poisoning  neuralgias  are  to  be  borne  especially  in  mind,  while 
diabetes  is  of  prime  importance.  Brachial  psychalgia  is  a  possibility, 
but  the  diagnosis  must  be  made  with  extreme  caution  after  a  rigid 
exclusion  particularly  of  organic  factors.  Hysterical  and  neurasthenic 
neuralgias  occur  in  this  distribution  as  well. 

Treatment. — Rest  is  a  necessity,  and  is  primarily  insured  by  means 
of  a  sling.  The  diagnosis  of  a  cause  being  assured,  treatment  should 
be  begun  to  remove  it,  either  by  medical  or  surgical  means.  Nerve 
suturing  for  injury  has  come  to  occupy  an  important  place,  and  is 
usually  attended  with  good  results,  even  after  long  periods  of  loss  of 
function.  In  the  early  stages  active  mechanical  treatment  is  to  be 
avoided.  Hot  applications  are  useful  in  most  acute  neuralgias.  In  the 
later  phases  massage,  particularly  the  Nageli  movements,  are  valuable. 
Galvanism,  3  to  6  milliamperes,  is  well  adapted  to  these  neuralgias, 
but  usually  much  better  results  are  obtained  by  the  Leduc  rapidly 
alternating  currents.  Salicylates  (especially  in  analgesic  combinations) , 
iodides,  quinin,  arsenic,  and  large  doses  of  strychnin  are  of  value  at 
times.  The  internal  secretions  are  curative  for  some.  Psychoanalysis 
is  to  be  used  in  hysterical  cases. 

Intercostal  Neuralgia. — ^The  twelve  dorsal  nerves  constitute  the 
plexus  involved,  although  the  upper  series,  especially  of  the  left  side, 
are  most  frequently  concerned.  Bernhardt  says  that  the  site  of 
election  is  mostly  from  the  fifth  to  the  ninth.  Since  the  dorsal  nerves 
divide  into  internal  and  external  branches  the  site  of  the  neuralgia 
may  be  on  the  surface  or  within  (pleurodynia,  etc.).  The  two  upper 
nerves  send  branches  to  the  internal  surface  of  the  arm,  and  pain  is 
occasionally  felt  there.  The  abdominal  involvements  are  rarer  and 
may  extend  down  to  the  genitals.     (See  Figs.  11  to  16.) 


2G4  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

Women  more  often  show  this  form  of  neuralgia  than  men,  and  the 
disorder  is  much  more  common  in  cold  weather. 

The  pains  are  usually  less  severe  than  in  other  regions,  although 
their  sharp,  sticking  character  distresses  the  chest  movements,  espe- 
cially since  all  movement  tends  to  aggravate  them.  Tender  points  are 
found  at  the  site  of  perforations  near  the  spine.  Skin  hyperesthesia  is 
extreme  at  times.  Herpetic  neuritic  neuralgias  are  relatively  common 
in  this  distribution. 

Among  the  causes  to  be  diagnosed  may  be  costal  caries,  affections 
of  the  spinal  cord  and  meninges,  disorders  of  the  pleura,  particularly 
carcinoma  and  tuberculosis,  aortic  aneurism,  dilatation  of  the  stomach, 
carcinoma  of  the  liver,  angina  pectoris,  pericarditis,  local  trauma, 
fractures,  etc. 

Mammary  neuralgia  or  mastodynia,  which  is  frequent  in  the  later 
stages  of  nursing,  and  in  some  women  at  the  menstrual  epoch,  is  a 
special  form.  The  pain  is  usually  deep  within  the  gland,  and  may  be 
accompanied  by  a  slightly  increased  secretion.  The  whole  skin  may  be 
sensitive,  especially  the  nipple,  when  the  superficial  nerves  are  mostly 
involved.  Local  glandular  induration  occasionally  occurs.  This  has 
led  to  the  mistaken  diagnosis  of  carcinoma,  but  a  neuralgia  may  occur 
due  to  a  carcinoma  of  the  breast.  Tabes  may  give  rise  to  an  intercostal 
neuralgia. 

Treatment. — Local  applications  are  useful,  especially  the  ethyl 
chloride  spray.  Blisters  are  efficacious.  Bandaging  affords  marked 
relief.    General  measures  already  described  call  for  no  further  mention. 

Lumbar  Plexus  Neuralgias. — These  are  most  conveniently  arranged 
as  (1)  lumbo-abdominal;  (2)  ilioscrotal  or  testicular,  (3)  crural  (scia- 
tica), (4)  femoral,  and  (5)  obturator,  involving  in  each  case  certain 
of  the  branches  of  this  plexus.  Mixed  and  indeterminate  forms  are  not 
infrequent. 

The  Sciaticas  (sciatic  neuralgias,  sciatic  neuritides). — It  has  already 
been  indicated  that  it  is  largely  indifferent  whether  one  regards  this 
as  a  neuralgia  or  a  neuritis,  since  transition  forms  are  very  frequent. 
It  consists  of  pain  in  the  distribution  of  the  nerves  of  the  sacral  plexus, 
the  sciatic  and  its  branches. 

History. — Cotugni,  in  1764,  gave  so  clear  a  description  of  sciatica 
that  the  malady  is  often  given  his  name.  Valleix,  in  1841,  described 
the  painful  points  with  great  minuteness.  Lasegue,  in  1864,^  described 
his  well-known  symptoms  of  neuritis  of  the  sciatic,  since  which  time 
many  monographs  have  appeared,  the  most  important  of  which  are 
those  of  Briihl,  Lago,  Vulpian,  and  Bernhardt. 

Etiology. — In  the  same  manner  that  one  finds  for  the  other  neural- 
gias and  neuritides,  similar  causes  are  at  work  for  sciatic  pains.  It  is 
unnecessary  to  amplify  these  causative  factors.  Any  of  those  general 
causes  found  on  the  previous  page  may  cause  a  sciatica,  but  special 

lArch.  Gen.  de  Med.  (1864).     Oppenheim. 


LUMBAR  PLEXUS  NEURALGIAS 


265 


emphasis  should  be  laid  upon  two  or  three.  Trauma  is  responsible 
in  many  cases  for  the  development  of  sciatica.  Syphilitic  osteo- 
arthritis, and  syphilitic  meningitis  of  mild  grade  are  responsible  for  the 
development  of  intractable  sciaticas.  Certain  French  authors  claim 
as  high  as  90  per  cent,  of  all  sciaticas  to  be  due  to  this  syphilitic  factor. 
In  this  respect  then  sciatica  stands  in  sharp  contrast  to  the  neuralgic 
neuritides  of  the  upper  extremities. 

Gout  is  an  infrequent  causative  factor,  while  diabetes  is  more 
common,  especially  for  double-sided  sciaticas.  Double  sciaticas  may 
also  be  the  expression  of  a  tumor  of  the  pelvis,  of  pressure  due  to  a 
gravid  uterus,  of  venous  stasis,  spinal  cord  tumor,  or  new  growths 
of  the  pelvis.     Occasionally  prostatic  enlargement  of  tuberculous  or 


ILIOHYPOGASTRIC 
ILIO-INGUINAL 


GENiTOFtMORAL 

EXTERNAL 
CUTANEOUS 


TO   PSOAS    AND 
ILIACUS 


iL  (anterior  r\         /ACCESSORY// 

crural)^V/    obtur«tor/,<        -- 
^^  obturator\\,X 

Fig.  128. — Diagram  of  the  lumbar  plexus. 


gonorrheal  origin  have  given  rise  to  sciatic  pains.  Exposure  to  cold, 
with  prolonged  standing  is  frequently  met  with  in  the  histories,  and 
occasionally  in  those  who  sit  a  great  deal.  Prolonged  walking  or 
marching  may  occasion  an  attack,  and  bicycling  predisposes  to 
mechanical  injury  of  the  nerve. 

It  is  a  comparatively  common  affection,  particularly  in  men,  being 
one  of  the  most  frequent  neuralgias  met  with  in  dispensary  practice. 
Chronic  constipation  as  a  cause  should  not  be  overlooked.  The 
etiological  factor  in  some  cases  is  impossible  to  find;  this  is  a  result 
of  our  insufficient  methods  of  examination. 

Symptoms. — There  is  no  one  sciatica,  there  are  many,  and  it  is 
advisable  at  the  outset  to  separate  those  cases  in  which  the  principal 


266 


AFFECTIONS  OF   THE  PERIPHERAL  NERVES 


symptoms  are  pain  and  inability  to  use  the  limb  from  those  in  which 
there  is  added  nerve  tenderness,  with  motor,  sensory  and  trophic 
phenomena. 

The  pains  rarely  commence  abruptly,  but  beginning  more  or  less 
gradually  from  a  sense  of  soreness  to  uneasiness  with  occasional 
twinges,  gradually  develop  into  well  marked  severe  pains,  usually  at 
first  more  intense  just  beneath  the  sciatic  notch,  gradually  extending 
from  above  downward  to  the  entire  distribution  of  the  sciatic  and 
some  of  the  branches.    There  is  usually  considerable  variation  in  the 


FOURTH  LUMBAR 


FIFTH  LUMBAR 


FIRST  SACRAL 


'SECOND  SACRAL 


THIRD  SACRAL 


FOURTH  SACRAL 


PERINEAL  BR.  TO  SPHINCTER 
V/'/'l/-''^         &  LEVATOR  ANI 

'.  FIFTH  SACRAL 


TO  COCCYGEUS 


.^y^-v--^:^,5  COCCYGEAL 
Fig.  129. — Plan  of  sacral  plexus  with  the  pudendal  plexus.     (Gerrish.) 


character  of  the  pain  during  an  attack.  Some  patients  suffer  for  some 
time  simply  from  boring,  dull  pains,  while  others  have  excruciatingly 
sharp,  stabbing  twinges  that  make  the  slightest  movements  impossible. 
Rarely  continuous,  the  pains  come  in  attacks,  sparing  almost  no  region 
of  the  distribution  of  the  plexus  either  en  masse  or  picking  out  special 
branches. 

The  pain  in  the  proximal  portions  of  the  leg  is  usually  deep  seated, 
but  becomes  more  superficial  distally.  In  some  patients  an  extensive 
series  of  involuntary  adaptive  positions  take  place  with  slight  scoliosis 
in  order  to  seek  the  most  comfortable  position,  not  only  of  the  thigh 


LUMBAR  PLEXUS  NEURALGIAS  267 

and  leg,  but  of  the  pelvis,  or  even  the  vertebral  column.  In  sitting, 
similar  adaptive  positions  are  taken  to  try  to  avoid  pressure. 

Painful  pressure  points  were  pomted  out  as  fairly  constant  by 
Valleix.  The  most  important  of  these  are  situated  at  the  sacroiliac 
joint,  the  sciatic  notch,  or  the  gluteal  point  on  the  gluteal  fold  over 
the  nerve,  and  the  peroneal  point  at  the  head  of  the  peroneus.  In  some 
cases,  often  mild,  pressure  points  are  lacking.  Lasegue's  phenomenon 
is  of  considerable  diagnostic  significance.  It  is  brought  out  either  in 
the  lying  or  sitting  position.  The  patient's  foot  is  grasped  with  one 
hand,  the  other  placed  upon  the  knee,  and  keeping  the  leg  stiffly 
extended  the  thigh  is  flexed  upon  the  hip,  when  a  severe  pain  develops 
beneath  the  knee  or  higher  up  in  the  course  of  the  nerve.  In  the  sitting 
position  the  pain  is  more  apt  to  be  beneath  the  knee,  since  the  extension 
on  the  pelvis  cannot  be  made  so  extreme. 

With  the  general  extension  of  the  neuritic  process — as  in  many 
alcoholic  cases — the  entire  nerve  may  be  sensitive  to  pressure. 

Minor^  has  described  some  interesting  findings  on  having  the 
patients  arise  from  a  prone  posture.  Patients  with  well-marked 
sciatica  can  rarely  get  up — without  excessive  pain — with  the  arms 
crossed.  They  put  their  two  hands  behind  them,  push  the  hips. back- 
ward, between  the  arms,  flex  the  knees  slowly  beneath  the  buttocks, 
then  gradually  with  one  hand  on  the  hips,  and  then  on  the  knee,  the 
other  balancing  in  the  air  gradually  come  to  a  standing  position.  The 
procedure  is  not  invariable,  but  is  useful  in  gaining  some  idea  as  to 
simulation,  and  as  to  the  diagnosis  of  a  lumbago.  The  untrained  simu- 
lator gets  up  in  a  variety  of  ways,  the  lumbago  patient  usually  rises  on 
all  fours,  the  arms  in  front,  somewhat  as  does  the  pseudohypertrophic 
dystrophy  patient. 

In  those  groups  of  patients  with  more  serious  neuritic  involvements 
one  has  added  the  signs  of  muscular  atrophy,  of  circumscribed  par- 
alyses, of  fibrillary  contractions,  of  contractures,  of  sensory  disorders, 
of  trophic  disturbances. 

The  atrophies  may  be  true  atrophies,  but  are  usually  position  or 
disuse  atrophies.  They  are  found  by  palpation,  or  by  measure- 
ments. 

Moreover  the  atrophies  may  be  segmentary,  or  radicular,  in  either 
of  which  instances  a  localizing  diagnosis  is  aided.  The  electrical  exam- 
ination of  the  muscles  in  sciatic  neuritis  is  usually  contradictory.  In 
positive  cases  with  partial  R.  D.  the  nature  of  the  process  becomes 
clearer.    Fibrillary  contractions  may  occur  in  the  paretic  muscles. 

The  patellar  reflex,  in  the  beginning  of  the  process,  is  usually  in- 
creased on  the  affected  side,  while  if  marked  neuritis  be  present  it 
may  be  diminished  or  absent.  The  Achilles  tendon  reflex  shows  a 
similar  reaction.  Oppenheim  has  called  attention  to  a  mild  degree  of 
flabbiness  or  diminution  in  the  size  of  the  Achilles  tendon  on  the 

1  Deut.  med.  Woch.,  1898. 


268  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

affected  side.      A  pseudo-Babinski   is   described,   largely   a   plantar 
flexion  of  the  smaller  toes,  with  immobility  of  the  great  toe. 

Sensibility  is  often  unaffected,  but  careful  testing,  following  Head's 
methods,  may  show  a  hypesthesia  to  touch,  pain,  and  temperature. 
Occasionally  the  disturbance  of  sensibility  shows  a  marked  radicular 
distribution,  which  speaks  for  a  more  or  less  localized  process  in  the 
plexus,  frequently  of  a  syphilitic,  meningomyelitic,  or  osteo-arthritic 
nature. 

Trophic  and  vasomotor  phenomena  are  not  infrequent,  consisting 
either  of  acroparesthesise,  erythemas,  local  cyanoses,  diminished  or 
increased  perspiration,  changes  in  the  growth  and  character  of  the 
hair  or  even  the  nails.  True  herpes  is  rare.  Glycosuria,  polyuria, 
azoturia  are  rare  complications. 

Course. — ^In  the  early  attacks  in  healthy  adults,  the  prognosis  is 
good.  The  patient  usually  recovers  in  from  six  to  eight  weeks  under 
proper  treatment.  A  failure  to  respond  should  excite  suspicion  as  to 
the  diagnosis,  especially  with  reference  to  tabes,  to  a  spinal  growth  or  a 
radiculitis.  Recurrent  cases  usually  develop  a  tormenting  chronicity, 
which  exhausts  one's  therapeutic  resources,  and  occasionally  drives 
the  patient  into  a  mental  state  which  suicide  or  chronic  morphinism 
alone  terminates.  Fortunately  such  cases  are  becoming  rarer  with 
better  methods  of  diagnosis  and  enlarged  therapeutic  resources. 

Clinical  Forms. — Certain  variants — largely  based  on  etiological 
concepts — may  be  met  with.  Fournier's  gonorrheal  sciatica  with  an 
acute  onset,  slight  temperature,  with  prostatic  and  articular  com- 
plications is  one.  Brissaud  has  described  a  spasmodic  type  with 
increased  tendon  reflexes,  contractures  in  the  periarticular  muscles 
of  the  hip,  and  trepidation  or  pseudoclonus.  Quenu's  varicose  scia- 
tica, which  has  certain  analogies  to  the  intermittent  claudication 
arteriosclerotic  type,  is  characterized  by  deep-seated  pain,  and  a  very 
protracted  onset  and  chronic  course.  Hysterical  sciaticas  may  always 
be  expected,  but  they  are  extremely  rare. 

Diagnosis. — The  increased  knowledge  afforded  by  lumbar  puncture, 
a;-rays,  and  finer  modes  of  testing  for  sensory  disturbances  is  dismem- 
bering the  old  sciatica  group  fairly  rapidly.  Of  the  more  common 
diagnostic  errors,  tabes  dorsalis  and  lumbago  call  for  special  mention. 

Tabes  lacks  the  pressure  points,  the  Lasegue  phenomenon,  and 
usually  shows  the  lost  knee-jerks,  lost  Achilles  jerk,  and  possible 
Argyll-Robertson  phenomenon.    The  pains  are  usually  bilateral. 

Lumbago  is  usually  much  relieved  by  the  recumbent  posture,  and 
is  increased  by  the  movements  of  the  trunk;  the  site  of  the  pain  is 
as  a  rule  higher. 

Lumbosacral  radiculitis  calls  for  special  mention  since  many  of 
the  classical  chronic  sciaticas  fall  under  this  disorder. 

Muscular  rheumatism  lacks  the  pressure  points,  Lasegue's  sign,  and 
the  ipains  are  more  diffuse. 

Spinal  cord  tumors  in  their  initial  symptomatology  cause  sciatic 


LUMBAR  PLEXUS  NEURALGIAS  269 

pains,  usually  bilateral,  occasionally  unilateral,  but  soon  careful 
sensory  examination  shows  anomalies,  special  localizing  anomalies, 
and  pareses  and  trophic  symptoms  point  to  a  severe  lesion  of  the  cord. 

Hip-joint  disease  lacks  the  classical  situation  of  the  pains.  Arthritis 
deformans  cases  with  sciatic  pains  show  diminished  power  of  abduction 
and  adduction  and  the  joints  are  painful. 

Intermittent  claudication  occasionally  gives  rise  to  diagnostic 
difficulties.  Its  arteriosclerotic  nature  is  revealed  by  palpation  of  the 
bloodvessels,  and  by  a--ray  examinations  which  show  the  tortuous 
modified  bloodvessels. 

Achillodynia,  Morton's  tarsalgia,  and  flat-foot  are  occasional  diffi-' 
culties. 

Therapy. — Rest  and  quiet  are  the  first  essentials.  Then  an  etiologi- 
cal therapy  becomes  imperative.  Mercury  for  syphilitic  cases,  quinin 
for  malarial  cases,  surgical  intervention  for  pressure  cases,  diet  for 
diabetic  cases,  etc. 

While  one  is  waiting  to  obtain  a  clear  notion  as  to  etiology,  general 
treatment  may  be  necessary.  Such  treatment  should  always  be 
regarded  as  provisional,  not  final. 

For  the  treatment  of  the  pain,  analgesics  are  imperative.  These 
are  numerous,  and  different  patients  will  respond  to  different  members 
of  the  group.  Even  during  an  attack  it  may  be  found  that  one  anal- 
gesic has  lost  its  value,  and  another  must  be  substituted.  It  is  of 
value  to  bear  the  chemical  structure  of  the  various  analgesics  in  mind 
in  one's  therapeutic  endeavors.  Antipyrin,  or  its  related  products, 
aspirin,  salipyrin,  pyramidon;  the  amido-phenol  series,  with  phenace- 
tin,  lactophenin,  exalgin,  apolysin,  citrophen,  phenosal,  phenocol,  and 
salocol,  as  representatives  are  often  of  signal  service,  but  require 
considerable  testing.  Acetanilid  itself,  with  its  compounds,  is  in  wide 
use.  They  have  active  analgesic  properties,  but  one  runs  the  risk  of 
cheap  and  impure  products,  since  acetanilid  is  so  extensively  manu- 
factured largely  as  a  by-product.  These  analgesics  can  be  used  in 
combination,  when  smaller  doses  of  the  two  or  three  in  use  are  more 
efficient  than  large  doses  which  often  have  marked  toxic  action,  either 
on  the  blood  cells  (acetanilid,  amido-phenol  series)  or  on  the  vaso- 
motors (antipyrin  derivatives). 

In  the  presence  of  anemia,  iron  and  arsenic  should  be  added. 

Counter-irritation  is  very  useful  especially.  It  is  best  practised  by 
means  of  the  actual  cautery  (Paquelin)  but  mustard  plasters,  canthar- 
ides  plaster,  etc.,  are  useful  adjuvants.  Applications  should  be  made 
along  the  nerve  trunks. 

Hydrotherapy  is  often  extremely  valuable  but  must  be  employed 
with  reason.  A  too  energetic  hydrotherapy  with  massage,  often 
aggravates  a  sciatic  pain,  especially  in  the  initial  stages  when  rest 
is  so  imperative.  Later  hot  packs,  mud  baths,  spray  douches,  with 
mild  massage  are  indicated.  In  many  patients  the  treatments  carried 
out  in  bath  resorts  is  especially  indicated.    Hot  air  treatment  is  not 


270  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

well  borne  in  the  initial  stages,  but  later  is  grateful  and  of  therapeutic 
value. 

Direct  nerve  injections  of  substances  having  a  degenerative  action 
in  nerve  fibers,  osmic  acid,  carbolic  acid,  etc.,  are  to  be  condemned. 
Infiltration  methods,  using  water  or  cocaine,  or  allied  substances,  or 
various  mixtures  have  more  to  recommend  them.  Schlosser  has 
reported  excellent  results  but  has  also  had  permanent  palsies  follow 
his  injections. 

At  times  it  may  be  deemed  necessary,  by  reason  of  the  severe  pain, 
to  practise  injections  of  stovaine,  cocaine  or  allied  substances  into  the 
region  of  the  cauda  or  into  the  spinal  cord  (Corning).  Such  injections 
are  useful,  but  their  action  is  temporary  as  a  rule.  Nerve  stretching 
is  to  be  condemned. 

The  opium  derivatives  should  be  used  only  as  a  last  resort. 

Electrotherapy. — The  older  methods  of  galvanization  and  faradiza- 
tion are  useful  in  a  few  cases,  but  on  the  whole  are  unsatisfactory. 
Sinusoidal  currents  are  more  valuable,  while  the  Leduc  rapidly  alter- 
nating currents  are  almost  always  of  some  service  in  relieving  pain 
but  not  in  curing.  High  frequency  currents  with  the  use  of  the  ultra- 
violet rays  at  times  give  extremely  satisfactory  results  froiji  the  same 
stand-point. 

Lumbo-abdominal. — These  occupy  the  lower  half  of  the  trunk,  and 
are  extremely  variable.  The  chief  nerves  involved  are  the  iliohypo- 
gastric and  its  branches,  the  inguinal,  and  genitocrural.  Strict  locali- 
zation to  one  trunk  is  rare,  and  men  are  more  frequently  affected  than 
women.  The  chief  causes  in  addition  to  those  of  general  moment 
are  local  inflammatory  conditions  or  new  growths  involving  the  plexus 
or  some  of  its  branches.  The  pains  are  usually  unilateral,  occasionally 
bilateral,  involve  the  region  of  the  back  below  the  ribs,  the  gluteal 
region,  the  abdominal  and  inguinal  areas,  the  scrotum,  or  the  labia. 
The  chief  Valleix's  points  are  over  the  lumbar  vertebrae,  the  hip  or 
iliac  point,  hypogastric  point,  and  the  scrotal  point.  Lumbo-abdom- 
inal pains  are  usually  accompanied  by  intercostal  pains  above  or 
thigh  pains  below. 

Testicular  Neuralgia.— Astley  Cooper  termed  this  neuralgia  the 
"irritable  testicle."  The  pains  are  usually  unilateral  and  pass  into  the 
testicle  which  may  be  swollen  and  tender  to  the  touch.  The  pain  not 
infrequently  passes  into  the  leg  and  back,  and  the  patient  may  have 
an  attack  of  vomiting.  Bernhardt  notes  that  the  pain  may  be  so  in- 
tense as  to  cause  the  patient  to  seek  castration.  The  affection  is  an 
obstinate  one,  and  is  not  helped,  as  a  rule,  by  removal  of  the  testicle. 
Diagnosis  involves  a  rigid  exclusion  of  organic  disorder  of  the  testicle 
although  many  affections  (gonorrhea,  tuberculosis,  chronic  prostatitis, 
etc.)  are  not  infrequently  accompanied  by  persistent  neuralgic  pains. 

Crural  Neuralgia. — The  crural  or  femoral  nerve  is  here  implicated. 
The  pain  extends  in  the  upper  front  and  inner  side  of  the  thigh,  to 
the  knee,  and  further  through  the  saphenous  distribution  to  the  ankle 


NEURALGIAS  OF  THE  PUDENDAL  PLEXUS  271 

and  inner  aspect  of  foot,  extending  as  far  as  the  big  toe.  It  is  almost 
entirely  confined  to  men,  and  shows  considerable  variability  as  to  the 
branch  involved.  It  not  infrequently  accompanies  a  sciatica.  Special 
etiological  features  are  found  in  fecal  impaction,  or  even  chronic  con- 
stipation, disease  of  the  hip  or  knee  bones,  enlargement  of  the  inguinal 
glands,  aneurism  of  the  iliac  artery.  Charcot  called  attention  to  the 
frequent  association  of  crural  neuralgia  and  diabetes.  Spinal  arthritis 
is  an  obscure  cause. 

Movements  of  the  thigh  usually  are  painful  and  the  patient  comes  to 
bend  his  body  forward  in  a  strained  position.  The  painful  points  of 
greatest  frequency  are  just  below  Poupart's  ligament,  just  within  the 
inner  condyle,  over  the  malleolus,  inner  side  of  the  instep,  and  one 
over  the  great  toe.  Neuro-atrophic  changes  usually  occur  in  the 
quadriceps,  but  the  patellar  reflex  is  rarely  affected,  save  when  a 
definite  neuritis  is  present.  Herpes,  reddening,  hyperesthesia  are  not 
infrequent.  In  the  diagnosis,  disease  of  the  inguinal  vessels  is  to  be 
looked  for,  as  well  as  intrapelvic  disorders,  new  growths,  etc.  Crural 
neuralgias  have  a  fairly  good  prognosis. 

Femoral  Neuralgia. — Here  the  cutaneous  femoris  lateralis,  arising 
higher  up  in  the  pelvis,  is  involved.  The  pain  is  felt  in  the  upper  and 
outer  aspects  of  the  thigh,  extending  to  the  knee.  A  painful  point  over 
the  anterior  spinous  process  of  the  ilium  is  usual.  Paresthesia  in  the 
distribution  of  this  nerve  has  been  extensively  studied  (Meralgia 
paresthetica) .  The  relation  of  the  pressure  of  corsets  in  the  causation 
of  this  type  of  neuralgia  has  been  pointed  out  by  Freud,  and  much  sit- 
ting in  adipose  individuals  is  frequently  associated  with  this  neuralgia. 
The  prognosis  is  favorable. 

Obturator  Neuralgia. — Lesions  of  this  nerve  are  fairly  constant  as  a 
result  of  the  pressure  of  the  intestinal  loops  of  a  hernia.  The  pain  is 
located  in  the  inner  side  of  the  thigh,  and  is  accompanied  by  a  feeling 
of  stiffness,  creepy,  crawly  feelings  of  the  skin,  and  inability  to  bring 
the  thigh  toward  the  middle  line  of  the  body. 

Neuralgias  of  the  Pudendal  Plexus. — A  large  number  of  neuralgias 
of  the  genital  plexus  are  recorded.  The  median  hemorrhoidal  branches, 
distributed  to  the  rectum,  bladder,  and  vagina,  the  inferior  branches 
to  the  anus,  and  the  pudendal  nerve  supplying  the  testicular  sac,  the 
labia,  penis,  urethra,  and  clitoris,  are  the  chief  nerves  involved.  The 
general  terms,  spermatic  neuralgia,  anal  neuralgia,  perineal  neuralgia, 
rectal  neuralgia,  vesical  neuralgia  or  cystalgia,  urethralgia,  prostalgia, 
penis  neuralgia,  irritable  uterus,  ovarian  neuralgia,  are  utilized  to 
describe  these  different  affections.  These  neuralgias  are  very  rare, 
but  often  very  obstinate.  Spermatic  neuralgias  are  among  the  most 
frequent,  and  are  not  infrequently  accompanied  by  painful  priapism, 
perhaps  ejaculation. 

Since  the  advent  of  bicycle  riding  neuralgias  of  this  general  region 
have  been  on  the  increase.  The  ovarian  neuralgias  are  complex,  and 
more  often  come  within  the  domain  of  the  gynecologist,  as  structural 


272  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

defects  are  usually  the  underlying  causes.  Localized  herpetic  eruptions 
accompany  neuralgias  of  this  plexus.  Lesions  of  the  cauda  equina 
are  to  be  carefully  excluded  in  neuralgias  of  this  region. 

Neuralgias  of  Coccygeal  Plexus. — Coccygodynia,  painful  coccyx,  is 
a  not  infrequent  disorder  in  women,  especially  in  multiparse  and  in  the 
badly  constipated.  Trauma  and  caries  are  frequent  causes.  The 
hysterical  coccyx  is  not  infrequent,  and  referred  coccygeal  pains  are 
common.  The  pain  is  so  intense  at  times  that  defecation  is  rendered 
impossible;  the  patient  cannot  sit,  and  a  grave  neurasthenic  condi- 
tion supervenes.  The  medicolegal  significance  of  coccygodynia  is  real, 
appearing  frequently  as  a  local  symptom  of  a  general  traumatic 
neurosis.  Surgeons  frequently  lay  considerable  stress  on  a  freely  mov- 
able coccyx  in  accident  litigation.  A  just  estimate  of  the  true  bearing 
of  an  injury  to  the  coccyx  can  only  be  arrived  at  by  a  careful  survey 
of  all  of  the  factors  of  the  particular  case. 

Local  treatment  is  seldom  efficacious  save  in  the  truly  neuralgic 
types.    Resection  is  rarely  a  justifiable  procedure. 

HERPES    ZOSTER:     SHINGLES:     ZONA.     RADICULO  GANGLIONIC 
SYNDROME  (ACUTE  POSTERIOR  POLIOMYELITIS). 

Li  a  broad  sense  zoster  consists  of  a  special  type  of  painful  erythe- 
matous eruption  with  formation  of  vesicles  following  the  radicular 
distribution  of  the  segment  involved,  due  to  disease  of  the  posterior 
roots  and  the  sensory  ganglion.  In  this  sense  it  may  be  due  to  an  acute 
or  chronic  meningitis,  tabes,  Pott's  disease,  carcinoma  of  the  vertebrae, 
acute  infectious  diseases,  intoxications  or  other  lesions  implicating 
the  posterior  roots  and  the  ganglion  (Symptomatic  Zoster) . 

In  a  narrower  sense  it  may  be  conceived  of  as  a  specific  infectious 
disease  affecting  the  ganglion  cells  in  the  posterior  spinal  ganglia 
and  the  adjacent  fibrillary  structures  (essential  zoster  or  posterior 
poliomyelitis) . 

History. — Zona  was  first  well  described  by  Rayer  in  1835,  although 
notes  on  its  occurrence  date  from  Hippocratic  times.  Baerensprung, 
in  1861,  gave  the  first  important  monograph,  and  pointed  out  the 
implication  of  the  ganglia  as  an  essential  feature  of  the  disease,  while 
Head  and  Campbell  (1900)  called  particular  attention  to  the  specific 
infectious  type.  Rosenow  and  OftedaP  have  isolated  streptococci  from 
the  ganglia. 

Etiology. — Nearly  all  of  the  general  causes  which  give  rise  to  a 
neuralgia  or  a  neuritis  may  by  an  extension  or  an  intensification  of  the 
pathological  process  involve  the  posterior  ganglia  and  thus  develop  a 
herpes.  In  poisoning  by  arsenic  and  carbon  monoxide  these  ganglia 
seem  to  be  specially  affected,  and  the  acute  gastro-intestinal  affections, 
pneumonia,  and  tuberculosis  are  not  infrequently  contributory  factors. 

1  Jour.  Amer.  Med.  Assoc,  June  12,  1915. 


HERPES  ZOSTER— SHINGLES— ZONA 


273 


Trousseau  first  called  attention  to  the  zoster,  which  was  a  specific 
infection,  which  type  has  been  so  extensively  studied  by  Head  and 
Campbell.     Epidemics  of  zoster  point  to  the  truth  of  this  position. 

Symptoms. — Neuralgic  pains  and  a  skin  eruption  constitute  the 
main  symptoms.  The  disorder  shows  a  slightly  different  order  of 
development  according  to  the  etiological  factors.  In  the  pure  or 
essential  zosters  (acute  posterior  poliomyelitis)  there  is  usually  a 
feeling  of  malaise,  a  slight  temperature,  and  gastro-intestinal  disturb- 
ances, then  the  patient  has  neuralgic  pains  which  may  be  mild  and 
superficial,  burning  or  pricking,  or  deep  and  extremely  severe,  and 
in  from  three  to  four  days  an  eruption  develops.     There  is  marked 


Fig.  130. — Herpes  zoster.    Typical  thoracic  location.     (Knowles.) 


hyperesthesia  of  the  skin  along  the  affected  segment,  with  redness,  and 
suddenly  or  gradually  there  appears  a  group  of  vesicles  varying  in  size 
from  a  few  millimeters  to  a  few  centimeters.  These  vesicles  are  rarely 
confluent,  and  the  fluid,  which  is  at  first  serous,  sometimes  tinged  with 
blood,  may  later  become  purulent.  The  vesicles  gradually  dry,  leaving 
a  scaly,  yellowish-brown,  stained  scar  which  persists  for  a  long  period. 
Ulceration  or  gangrene  occasionally  occurs.  The  cycle  occupies 
about  four  to  eight  days.  There  is  usually  some  anesthesia  to  both 
epicritic  and  protopathic  sensibility  after  the  acute  stage  has  passed. 
One  attack  seems  to  confer  immunity. 

In  the  symptomatic  zosters  there  is  rarely  fever  or  gastro-intestinal 
disturbance,  the  development  of  the  eruption  is  usually  irregular,  and 
18 


274  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

it  often  shows  a  chronic  character.  Symptomatic  zona  may  involve 
both  sides,  whereas  the  infectious  type  is  practically  invariably  one- 
sided. 

In  the  dorsal  types  only  is  the  girdle  distribution  maintained  (inter- 
costal herpes  zoster),  whereas  involvement  of  the  Gasserian,  cervical, 
lumbar  or  sacral  roots  gives  rise  to  irregular  eruption  appearances  by 
reason  of  the  segmental  complexities  of  these  regions. 

Ophthalmic  zoster  is  an  especially  severe  type,  occurring  in  individ- 
uals above  middle  life,  and  often  accompanied  by  alcoholic  and  arterio- 
sclerotic factors.  It  may  develop  apparently  like  an  erysipelas  of  the 
face,  with  severe  neuralgia,  and  then  a  widespread  vesicular  eruption, 
even  involving  the  mucous  surface,  develops.  Ocular  complications, 
conjunctivitis,  keratitis,  iritis,  of  a  severe  nature,  are  not  infrequent. 
Facial  palsy  may  be  present  in  zoster  of  the  Gasserian  ganglion  or  of  the 
geniculate.  A  symptomatic  ophthalmic  zoster  due  to  lesions  in  the 
region  of  the  pons  occurs. 

Pathology. — In  essential  zona  there  is  an  acute,  often  hemorrhagic 
inflammation  in  the  sensory  ganglia.  These  are  swollen,  the  capsule 
notably  thickened,  with  marked  infiltration  of  leukocytes.  The 
ganglion  cells  are  in  part  destroyed  or  damaged,  and  the  contiguous 
fibrillary  structures,  both  central  and  peripheral,  are  also  involved 
in  the  inflammatory  reaction.  The  inflammation  is  usually  limited 
to  a  few  ganglia.  In  the  spinal  cord  secondary  degenerations  have 
been  observed,  and  occasionally  there  is  an  extension  of  the  general 
process  to  the  cord.  In  certain  cases  of  what  appears  to  be  essential 
zona  the  ganglia  have  been  free,  the  only  lesions  found  being  those  of 
a  neuritis.  There  is  an  inconstant  lymphocytosis  of  the  cerebro- 
spinal fluid  in  the  infectious  zonas. 

Treatment. — For  the  symptomatic  cases,  the  cause  must  be  found. 
Otherwise  the  treatment  is  purely  symptomatic.  Local  applications 
of  zinc  oxide  ointment  for  protection  and  the  use  of  mild  antiseptic 
to  prevent  suppuration  are  advisable.  For  the  pains  the  analgesic 
remedies  already  spoken  of  in  the  treatment  of  neuralgia,  are 
useful.  Gastro-intestinal  therapy  relieves  the  discomfort  and  itching 
somewhat,  and  may  possibly  limit  the  accumulation  of  a  possible 
secondary  irritant. 

RADICULITIS. 

The  radicular  syndrome,  often  confused  with  neuralgia  and  neuritis, 
is  due  to  an  inflammation  of  the  sensory  nerve  roots,  usually  of  the 
brachial,  and  of  the  lumbosacral  plexuses. 

Attention  has  been  given  to  it  chiefly  by  French  neurologists, 
notably  Dejerine  (1905)  and  his  pupils.^ 

Symptoms. — These  are  neuralgic  pains,  which  are  usually  severe, 
yet  very  variable.     They  usually  occur  in  crises,  and  are  sticking 

1  Dejerine  et  Thomas.     Maladies  de  la  moelle  epiniere,  1909. 


RADICULITIS 


275 


and  lancinating  in  character,  at  times  extremely  intense;  not  infre- 
quently resembling  the  pains  of  tabes.  The  pains  have  a  tendency 
to  remit  and  then  to  recur  at  shorter  intervals,  leaving  a  certain 
soreness  behind.  The  nerve  trunks  are  usually  not  markedly  painful. 
There  is  usually  a  marked  hyperesthesia  over  the  radicular  segment, 
It  does  not  follow  the  peripheral  distribution  as  in  a  non-radicular 
neuralgia.  This  hyperesthesia  is  usually  followed  by  an  anesthesia 
to  both  epicritic  and  protopathic  sensibility,  and  occasionally  bony 
sensibility  is  involved  as  well  if  the  inflammatory  reaction  is  intense. 
Deep  sensibility  may  be  so  involved  as  to  cause  astereognosis. 
Paresthesiae  and  acroparesthesise  are  common. 


Figs.  131  and  132. — Topography  of  the  sensory  disturbance  in  a  syphilitic  radiculitis 
(type  Klumpke).    The  Cs  and  Di,  2  distribution  are  involved.    (Dejerine.) 


It  is  essential  that  these  features,  which  may  be  found  in  other 
affections,  be  radicular  in  their  distribution.  They  are  not  segmentary, 
i.  e.,  involving  the  hand,  the  forearm,  or  the  arm;  nor  do  they  follow 
the  peripheral  nerve  distribution.  They  are  distributed  in  long  bands 
down  the  arm  or  the  leg,  corres'ponding  to  the  root  segments  involved. 
(See  Figs.  133,  134,  and  also  Figs.  11  to  16,  and  Plates  X  and  XI). 

It  is  a  striking  fact  that  sneezing  or  coughing  may  bring  on  a  par- 
oxysm of  pain  in  the  cervicodorsal  plexus,  and  coughing  and  straining 
at  stool  may  bring  on  pain  in  a  sciatic  radiculitis. 

The  knee-jerks  are  primarily  exaggerated,  later  diminished  or  lost. 

In  the  upper  extremity  it  is  rare  to  find  a  pure  radiculitis,  i.  e.,  one 


276 


AFFECTIONS  OF   THE  PERIPHERAL  NERVES 


without  some  motor  involvement,  while  for  the  lower  limb  the  great 
majority  of  the  classical  cases  of  sciatica  are  due  to  a  radiculitis. 

In  the  mixed  cases,  involvement  of  the  anterior  roots  causes  muscular 
atrophies,  likewise  radicular  and  not  peripheral  in  their  distribution. 
The  atrophy  is  rarely  accompanied  by  fibrillary  twitchings,  nor  by 
spasmodic  contractions. 

Ataxias  may  be  met  with.  Lasegue's  sign  is  commonly  found 
(Rousellier,  Paris  These  1907).    Vallieux's  points  are  not  constant. 


Figs.  133  and  134. — Scheme  of  root  (radicular)  segment  distribution. 
(Compare  with  Figs.  19  to  22.) 


(Flatau.) 


Trophic  disturbances,  particularly  in  the  joints,  and  vasomotor 
signs  (cyanosis)  are  found  in  long-standing  cases.  In  rare  instances 
the  anterior  roots  are  involved  primarily. 

Diagnosis. — ^Lumbar  puncture  may  reveal  a  lymphocytosis.  As 
noted,  radiculitis  of  the  lumbosacral  plexus  has  for  the  most  part  been 
grouped  with  the  sciatic  neuralgias;  both  under  the  so-called  true  and 
the  symptomatic  neuralgias. 

With  extremely  careful  sensory  tests,  following  the  procedures  of 
Head  and  Dejerine,  the  radicular  distribution  of  the  hyperesthesise, 
the  anesthesiae  or  the  atrophies  is  sufficient  to  determine  a  diagnostic 


RADICULITIS  277 

picture.  The  crural  nerve  is  more  often  involved  in  the  radicular 
process  than  in  the  peripheral  sciaticas.^ 

The  presence  of  ataxia  in  the  lower  limbs,  occasionally  in  the  upper, 
Romberg's  sign,  and  the  frequent  loss  of  the  knee-jerks  often  leads  to 
the  mistaken  diagnosis  of  tabes.  From  one  point  of  view  tabes  begins 
as  a  radiculitis,  and  the  diagnostic  difficulty  centers  about  the  etio- 
logical element.  Since  so  much  of  radiculitis  of  the  lower  extremity 
is  due  to  syphilis,  the  ordinary  lumbar  puncture  and  Wassermann  tests 
are  essential  to  make  the  diagnosis  positive.  In  tabes  one  must  also 
take  into  consideration  the  involvements  of  the  cranial  nerves,  Argyll- 
Robertson  pupil,  etc. 

Cervical  radiculitis  naturally  travels  under  the  guise  of  a  cervico- 
brachial  neuralgia.  Here  the  greater  implication  of  the  sensory  system 
is  enough  to  make  a  diagnosis.  In  mild  early  cases  the  diagnosis  of  a 
radiculitis  is  impossible.  Muscular  atrophy  is  a  frequent  complication 
of  cervical  radiculitis.  Furthermore,  pupillary  phenomena  (Klumpke) 
are  common  in  this  type. 

Brachial  radiculitis  gives  rise  at  times  to  a  typical  Aran-Duchenne 
atrophy — the  biceps,  anterior  brachial,  supinator  longus  and  deltoid — 
the  fifth  and  sixth  cervical  roots  being  chiefly  involved.  The  Klumpke 
oculopupillary  phenomena,  i.  e.,  diminution  in  size  of  the  palpebral 
fissure,  slight  retrogression  of  the  eyeball  and  miosis  are  present  if 
the  last  cervical  and  first  dorsal  roots  are  involved. 

Disseminated  types  are  described  by  Dejerine^  with  practically 
all  the  signs  of  a  tabes. 

Occasionally  cerebral  tumors  cause  the  symptoms  of  a  radiculitis 
with  alterations  in  the  posterior  columns  and  radicular  lesions  (Collier, 
1899;  Nageotte,  1909;  Raymond,  1907).  Both  limbs  may  be 
involved. 

Acroparesthesia  was  originally  described  by  Putnam  (1882),  named 
by  Schultze,  and  then  shown  by  Pick  to  be  due  to  a  radicular  or  intra- 
spinal involvement,  chiefly  in  the  lower  half  of  the  cervical  region. 

It  consists,  in  the  periodic  cases,  of  pains  or  burning,  or  cold  sensa- 
tions, usually  in  the  fingers,  accompanied  by  blanching  and  coldness 
of  the  skin  with  the  sensation  of  engorgement  and  extreme  heaviness 
of  the  hand  or  the  fingers. 

Treatment. — Spontaneous  radiculitis  seems  to  be  preeminently 
syphilitic.  Hence  an  antisyphilitic  treatment  is  indicated  in  every 
case  of  suspected  radiculitis.  Other  forms  of  meningitis  may  produce 
it,  however.  The  prognosis  is  good  in  the  syphilitic  forms,  but  less 
so  for  the  others.  Many  patients  with  cervical  and  brachial  radicu- 
litis recover  spontaneously,  after  from  six  to  ten  months.  Treatment 
seems  to  alleviate  but  not  cure.  The  general  treatment  for  a  neuritic- 
neuralgia  is  indicated.  Violet-ray  exposure  should  be  tried  in  the 
intractable  cases. 

1  Dejerine,  Semiologie  du  systeme  nerveux,  1914. 

2  Rev.  Neurol.,  1904,  p.  524. 


278  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

NEURITIS. 

Neuritis  is  a  generalized  inflammation  of  the  peripheral  nerves 
involving  in  varying  degrees  of  completeness  the  motor,  sensory  and 
trophic  elements.  '  In  old-standing  cases  the  spinal  portions  of  the 
neurones  are  implicated. 

The  inflammatory  changes  may  proceed  either  from  the  peri- 
neurium, from  the  endoneurium  or  involve  the  axis  cylinder.  These 
may  be  poisoned  and  degenerate  (parenchymatous  inflammation). 
The  pathological  nature  of  the  lesion  bears  little  relation  to  the  general 
symptomatology,  and  pathologically  considered  there  is  considerable 
interplay  of  the  various  processes. 

Etiology. — A  vast  variety  of  causes  may  bring  about  a  neuritis, 
seen  either  as  the  result  of  acute  toxic  parenchymatous  changes,  acute 
degeneration  due  to  actual  pressure  or  injury,  or  some  acute  or  chronic 
inflammatory  changes  following  a  variety  of  noxa.  The  most  important 
of  these  causes  are  as  follows : 

Infections. — Neuritis  may  result  from  the  toxins  of  microorganisms, 
as  those  of  diphtheria,  tuberculosis,  syphilis,  influenza,  smallpox, 
dysentery,  typhoid  fever,  pneumonia,  streptococcus,  occasionally 
measles,  scarlet  fever,  influenza,  whooping-cough,  etc.  Practically 
there  is  no  infectious  disease  that  has  not  produced  a  toxic,  usually 
parenchymatous,  neuritis.  A  second  group  of  infections,  such  as 
leprosy,  beri  beri,  malaria,  rabies,  bring  about  neuritis,  but  here 
the  mechanism  is  different. 

Intoxications. — Next  in  general  importance  are  the  intoxications, 
either  exogenous  or  endogenous.  Alcohol  plays  the  chief  role,  while 
arsenic,  lead,  carbon  monoxide,  sulphur,  and  anilin  compounds  are 
rarer  exciting  causes.  Of  the  auto-intoxications  diabetes  is  perhaps 
the  most  important.     Others  are  gout,  leukemia,  and  anemia. 

Trauma. — ^A  third  important  cause  for  the  neuritic  process  is  trauma 
to  the  nerve,  either  as  the  result  of  accident  or  injury,  or  from  the 
pressure  of  new  growths,  dislocations,  false  positions  or  other  anomalies 
of  structure  combined  with  the  practice  of  certain  occupations  (pro- 
fessional neuritis). 

Less  important  causes,  numerically  speaking,  are  extensions  of 
inflammatory  processes  (ascending  neuritis),  inflammations  above 
joints,  inflammations  of  adjacent  organs,  etc. 

Symptoms. — ^The  symptomatology  of  neuritis  varies  enormously,  not 
only  so  far  as  the  etiological  factors  which  determine  general  trends 
of  reaction  are  concerned,  but  also  with  reference  to  location,  acuteness 
of  onset,  etc. 

Only  the  symptoms  of  the  generalized  process  will  be  considered 
here,  reserving  for  the  sections  on  Pareses  or  Paralyses,  both  of  the 
plexuses  and  of  the  peripheral  nerves  a  more  detailed  description  of  the 
various  isolated  types. 

It  has  already  been  indicated  that  the  conceptions  neuralgia,  radicu- 


NEURITIS  279 

litis  and  neuritis  are  very  flexible — it  is  only  for  the  sake  of  description 
that  one  draws  more  or  less  arbitrary  lines  between  them.  Such  do  not 
exist  in  nature. 

There  is  a  generalized  type  of  neuritis  which  of  and  by  itself  con- 
stitutes a  fairly  definite  syndrome.  This  is  so-called  multiple  neuritis, 
or  polyneuritis.  It  is  largely  due  to  toxemias,  either  of  organic  or 
inorganic  nature. 

Under  the  general  caption  of  peripheral  neuritis,  one  considers  a 
large  number  of  peripheral  palsies,  while  as  localized  neuritis  one  has 
to  consider  a  number  of  the  professional  neurit  ides. 

Polyneuritis,  Multiple  Neuritis. — This  is  a  general,  widely  distrib- 
uted, diffuse,  parenchymatous  neuritis  in  which  the  entire  peripheral 
neuron  is  involved.  Secondary  degenerations  of  the  cerebral  neurons 
take  place. 

Etiolofy. — Multiple  neuritis  is  almost  invariably  due  to  some 
toxemia.  Such  toxemias  may  be  due  to  (1)  alcohol,  lead,  arsenic, 
carbon  monoxide,  bisulphide  of  carbon,  sulphuric  acid,  alcohol,  and 
some  of  the  rarer  metals:  mercury,  copper,  phosphorus,  etc.,  (2)  or 
to  the  toxins  of  acute  or  chronic  infectious  diseases,  such  as  smallpox, 
typhoid  fever,  grippe,  measles,  scarlet  fever,  diphtheria,  pneumonia, 
dysenteries,  streptococcemias,  leprosy,  tuberculosis  and  syphilis,  or 
the  inflammation  may  result  from  (3)  auto-intoxications  such  as  dia- 
betes, leukemias,  severe  anemias,  etc.  Acute  chilling  of  the  body  is 
held  to  be  responsible  for  certain  cases,  particularly  in  the  presence 
of  some  infectious  diseases,  or  acute  toxemias,  notably  in  alcoholic 
cases,  cases  of  rabies,  influenza,  etc. 

Occurrence. — No  general  laws  can  be  made  with  reference  to  occur- 
rence because  of  the  wide  range  of  etiological  factors.  Thus,  factors 
of  age,  of  social  state,  of  occupation,  etc.,  which  may  co-exist,  for 
instance,  with  the  occurrence  of  diphtheria,  have  nothing  whatever  to 
do  with  the  same  class  of  facts  in  cases  of  alcoholic  polyneuritis. 

Symptoms.^ — Clinically  considered,  one  meets  with  subacute  and 
acute  cases.  In  the  subacute  cases,  which  are  in  general  milder,  the 
patient  usually  begins  to  have  a  rapid  progressive  enfeeblement  of  the 
muscles,  as  a  rule,  of  the  lower  extremities.  There  is  rarely  any  fever 
at  the  onset,  and  the  loss  of  power  gradually  extends  from  the  periph- 
eral segments  toward  the  trunk.  Thus,  the  extensors  of  the  leg 
and  of  the  foot  first  show  weakness,  and  later  those  of  the  thigh  and 
hip.  At  the  same  time,  or  closely  following,  the  upper  extremities 
may  be  involved,  in  accordance  with  the  same  general  law,  the  muscles 
of  the  hand,  wrist  and  forearm  usually  being  primarily  involved. 
There  are  occasional  exceptions  to  this  general  law  of  progression,  but 
they  are  comparatively  rare. 

It  is  further  characteristic  that  the  weakness  and  paralysis  are  more 
or  less  symmetrically  distributed.  Although  one  leg  or  one  arm  may 
show  a  greater  amount  of  weakness  than  the  other  there  is  almost 
invariably  quadrilateral  involvement.     In  the  milder  cases  quantita- 


280  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

tive  variations  in  the  severity  occur,  and  in  the  mild  subacute  cases 
the  cranial  nerves  are  less  often  diseased.  Still  the  muscles  of 
the  abdomen,  the  diaphragm,  the  face,  eyeballs  or  tongue  may  all 
suffer. 

In  the  more  severe  cases,  the  implication  of  the  pneumogastric  is 
shown  by  tachycardia,  dyspnea  and  feebleness  of  the  pulse. 

The  superficial  reflexes  may  first  be  exaggerated,  but  later  become 
lost  as  a  rule,  and  the  tendon  reflexes  usually  exhibit  the  same  phenom- 
enon. Disturbances  of  sensibility  are  usually  more  marked.  Initial 
pain  is  more  or  less  universal,  but  the  sensations  of  actual  pain  are 
often  preceded  by  tingling  or  creeping  sensations,  and  the  skin,  muscles, 
nerve  trunks  and  joints  may  all  show  hyperesthesise.  The  Lasegue 
phenomenon  is  universally  present. 

Careful  testing  of  epicritic  sensibility  may  show  no  loss,  although 
as  a  rule  the  sense  of  localization  to  light  touch  and  the  ability  to 
distinguish  between  two  points  of  a  compass  soon  becomes  some- 
what diminished.  A  certain  amount  of  loss  of  epicritic  temperature 
sense  may  also  be  met  with.  In  the  milder  cases  the  atrophy  gradually 
disappears,  and  there  is  no  tendency  to  the  development  of  contract- 
ures, but  in  other  cases  contractures  may  follow,  and  the  limbs 
become  fixed  and  immobile. 

In  acute  generalized  polyneuritis  the  attack  begins  very  abruptly, 
usually  with  high  temperature  and  chill,  headache,  malaise,  suppression 
of  urine,  albuminuria,  and  the  general  signs  of  an  acute  illness.  Par- 
alyses develop  very  rapidly,  usually  involving  the  lower  extremities  first, 
and  gradually  ascending  the  trunk  and  the  arms,  closely  resembling 
the  ascending  type  of  acute  anterior  poliomyelitis  (Landry).  There 
is  great  tenderness  on  pressure  over  the  nerve  trunks,  sharp,  shooting 
pains,  marked  hyperesthesia  of  the  muscles  and  the  Lasegue  phenom- 
enon. The  reflexes  are  rapidly  abolished,  the  patient  loses  epicritic 
sensibility,  is  unable  to  localize  touch,  cannot  distinguish  points  of 
the  compass,  but  rarely  loses  sense  of  pain  or  of  deep  pressure.  The 
sphincters  are  not  usually  involved,  except  in  extremis.  Atrophies, 
contractures,  trophic  disturbances  of  the  skin,  such  as  glossy  skin, 
pemphigus-like  eruptions,  perforating  ulcers,  fragile  nails,  thick  and 
fragile  hairs,  etc.,  develop.  Then  ocular  palsies  are  met  with,  occa- 
sionally facial  palsy,  tinnitus  frequently  results  from  cochlear  disturb- 
ance, and  neuritic  vertigo  from  vestibular  disorder  is  found.  Pupillary 
inequalities  are  frequent  in  the  severe  cases.  Sluggish  light  and 
accommodation  reflexes  are  fairly  constant  and  occasionally  a  true 
Argyll-Robertson  pupil  is  found.  Loss  of  accommodation  with 
retained  light  reflexes  is  met  with  occasionally.  Amaurosis,  complete 
or  partial,  is  not  infrequent.    (See  Interstitial  Optic  Neuritis,  p.  189.) 

The  cranial  nerve  nuclei  are  not  infrequently  involved.  In  the  fatal 
cases  the  implication  of  the  pneumogastric  causes  death.  The 
symptoms  are  those  of  asphyxiation,  or  with  cardiac  irregularity  and 
paralysis  of  that  organ. 


MULTIPLE  NEURITIS  281 

Course. — Diagnosis.  —  Treatment.  —  Inasmuch  as  multiple  neuritis 
varies  not  only  with  reference  to  its  symptomatology  and  course,  but 
offers  special  diagnostic  problems  according  to  the  etiological  factors, 
and  since  the  treatment  must  depend  upon  a  due  consideration  of  the 
etiological  factors,  it  is  best  to  discuss  these  problems  under  special 
heads. 

Alcoholic  Multiple  Neuritis. — This  is  the  most  common  of  all  the 
types  of  multiple  neuritis.  Any  form  of  alcohol-containing  drink  can 
cause  it,  including  beer.  Cologne,  ether  and  other  substances  of  the 
marsh-gas  series  are  among  the  etiological  curiosities  as  causing 
neuritis. 

It  is  usually  subacute  in  development,  although  occasionally, 
especially  if  the  patient  has  been  subjected  to  severe  cold,  the  disease 
may  begin  in  a  very  acute  manner,  and  present  the  picture  of  a  Landry 
syndrome.  In  a  few  instances  an  apoplectiform  onset  has  been  noted. 
Intercurrent  infectious  disease  in  an  alcoholic  may  constitute  the  point 
of  departure  for  a  polyneuritis;  this  is  especially  true  of  influenza.. 

These  patients  usually  complain  of  tingling  sensations;  of  formica- 
tion over  the  hands  or  down  the  legs,  with  occasional  twinges  of 
pain,  particularly  in  the  legs.  The  skin  is  usually  hyperesthetic 
throughout  the  entire  body,  and  the  muscles  become  very  sore.  Such 
symptoms  may  be  present  for  weeks  or  even  months.  The  patient 
commences  to  notice  difficulty  in  walking,  inability  to  go  up  and  down 
stairs  and  occasional  falling  when  called  upon  for  certain  muscular 
exertion. 

Examination  of  the  muscular  power  at  this  stage  shows  marked 
weakness,  particularly  in  the  extensors  of  the  feet,  and  of  the  hands. 
In  walking  there  is  a  tendency  to  "drop-foot,"  and  the  patients  lift 
the  legs  a  little  higher,  and  may  show  a  characteristic  flopping  step 
superficially  resembling  that  of  the  tabetic. 

In  this  stage,  before  patients  are  confined  to  bed  by  reason  of  the 
muscular  weakness,  an  exaggeration  of  the  tendon  reflexes  is  occasion- 
ally found,  although  diminution  and  loss  becomes  the  rule.  Atrophy, 
flabbiness  of  the  muscles,  and  the  trophic  signs  may  then  appear. 

A  number  of  these  patients  are  able  to  be  about,  and  are  often 
mistaken  for  cases  of  beginning  tabes,  especially  as  there  is  very 
frequently  a  certain  amount  of  ataxia,  definitely  marked  in  the  lower 
extremities,  less  so  in  the  upper.     The  sphincters  are  as  a  rule  intact. 

In  some  severe  cases,  in  addition  to  the  physical  signs,  a  very  definite 
psychosis  develops.  This  is  treated  under  the  head  of  polyneuritic 
psychosis — chronic  alcoholic  delirium,  Korsakow's  psychosis.  (See 
chapter  on  Toxic  Psychoses.) 

Course. — Alcoholic  polj^neuritis  shows  an  innumerable  number  of 
varieties,  but  in  the  main  it  runs  a  subacute  course.  The  patients 
go  through  the  usual  symptoms  of  chronic  alcoholism,  with  tremor, 
sleeplessness,  gastric  disturbance  and  malnutrition.  In  the  severe 
cases  which  develop  marked  mental  signs — Korsakow's  syndrome — 


282 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


they  usually  go  through  one  or  more  periods  of  acute  delirium  (delirium 
tremens) . 

Then  the  neuritic  symptoms  commence  to  appear,  usually  with 
formication  or  other  paresthesise  in  the  arms  and  legs.  Sudden  twinges 
of  pain,  particularly  in  the  lower  limbs,  are  frequent,  and  an  unusual 
sense  of  muscular  fatigue  develops.  Dynamometer  readings  show  this 
loss  of  power  and  excessive  fatigability  early  in  the  disorder.  The 
average  case,  if  drinking  continues,  and  the  malnutrition  and  insomnia 
are  not  overcome,  takes  three  or  four  to  eight  weeks  for  the  develop- 
ment of  definite  palsies.  The  patients  note  great  difficulty  in  going 
up  and  down  stairs,  with  much  palpitation  and  shortness  of  breath. 
Then  on  some  sudden  exertion  they  fall,  or  their  legs  shut  up  like  a 
jack-knife  under  them.  They  totter  in  their  walk,  or  show  a  steppage 
or  ataxic  gait.    The  disease  may  be  arrested  at  this  stage,  and  recovery 


Fig.  135. — Alcoholic  neuritis  in  chronic  stage  with  contractures. 


takes  place  with  proper  care.  In  advancing  cases,  locomotion  becomes 
impossible.  Foot-drop,  wrist-drop,  and  ptosis  may  develop,  and  the 
atrophies,  contractures,  and  trophic  disturbances  come  on  rapidly. 
The  pains  have  increased  in  severity  and  frequency,  and  are  apt  to 
be  excruciating.  The  hyperesthesia,  which  has  been  excessive,  may 
now  be  gradually  and  irregularly  supplanted  by  hypesthesia  or  anes- 
thesia, hypalgesia,  or  analgesia,  and  extension  to  the  cranial  nerve 
nuclei  may  be  looked  for.  Sensitive  nerve  trunks  and  Lasegue's 
phenomenon  are  invariably  present. 

The  patient  who  has  advanced  to  the  stage  of  paralysis,  atrophies, 
and  trophic  changes  is  usually  confined  to  bed  for  several  months, 
and  then  commences  to  make  a  slow  and  irregular  recovery.  It  may 
be  complete,  but  there  is  apt  to  be  some  local  persistent  impairment 
which  may  require  treatment  for  years,  especially  if  fibrotendinous 


LEAD  NEURITIS  283 

contractures  have  developed.  In  the  Korsakow  cases  certain  grades 
of  residual  mental  impairment  are  extremely  common. 

No  two  cases  of  alcoholic  polyneuritis  are  alike.  There  is  a  general 
tendency  for  the  disorder  to  involve  all  four  extremities  in  the  pro- 
nounced cases,  but  the  lower  extremities  are  more  severely  implicated. 
Hyperesthesise  and  paresthesise  are  frequent.  Special  predilection  is 
shown  for  the  extensors  of  the  foot  and  wrist.  Pains  are  extremely 
severe  and  are  universal.  Blindness  (amaurosis)  is  frequent;  with 
methyl  (wood)  alcohol  it  usually  comes  on  in  advance  of  any  other 
neuritic  symptoms. 

Treatment. — ^Treatment  of  alcoholic  neuritis  involves  the  absolute 
withdrawal  of  alcohol  in  any  form,  complete  rest,  forced  feeding, 
particularly  with  foods  rich  in  fat — milk,  eggs,  butter.  Pain  is  best 
relieved  by  hot  applications;  continuous  hot  baths  are  very  grateful 
if  there  is  marked  hyperesthesia  of  skin,  muscles,  and  nerve  trunks; 
the  temperature  should  not  exceed  96°  to  97°  F.,  if  the  bath  is  to  be 
continued  for  any  great  length  of  time.  Active  catharsis  and  diapho- 
resis are  essential  in  the  early  stages.  After  the  stage  of  acute  hyperes- 
thesia is  passed  the  forced  feeding  should  be  continued  and  the  use  of 
strychnine  and  electricity  commenced.  Great  care  should  be  taken 
in  the  selection  of  the  hypnotics  used  to  give  sleep,  since  so  many  of 
them  contain  alcohol,  and  a  few  are  directly  poisonous  to  the  nerve 
trunks.  Bromides,  hyoscin,  or  occasionally  the  opium  derivatives 
may  be  employed.  If  an  alcohol  hypnotic  seems  necessary  paralde- 
hyde and  chloral  are  indicated. 

Glycerophosphites  with  calcium  are  valuable,  preferably  not 
given  in  an  alcoholic  medium.  Massage  and  muscular  movements  of 
various  types  are  indicated  in  the  chronic  stages,  and  most  cases  will 
repay  constant  working  upon  them.  In  the  presence  of  contractures, 
surgical  intervention  may  be  necessary,  but  should  be  deferred  until 
persistent  massage  and  muscular  therapy  have  been  exhausted. 

Lead  Neuritis. — Lead  Palsy. — In  neuritis  from  lead  poisoning  which 
occurs  from  the  gradual  intake  of  lead  in  some  form — drugs,  hair 
dyes,  cosmetics,  contaminated  water,  certain  occupations — plumbers, 
workers  in  type  foundries,  printers,  glaze  workers,  potters,  etc. — the 
picture  is  very  different  from  that  seen  in  alcoholism,  although  the 
terminal  stages  may  be  similar..  The  histological  alterations  are 
practically  identical.    Many  cases  are  complicated  with  alcoholism. 

Symptoms. — There  are  the  initial  gastro-intestinal  symptoms  of 
lead  poisoning,  furred  tongue,  constipation,  attacks  of  colic,  headaches, 
anemia,  painful  joints,  and  perhaps  the  signs  of  a  nephritis.  The  gum 
lead  line  is  frequently  present.  After  a  few  months,  or  even  a  year  or 
so  of  exposure,  the  neuritis  develops,  often  after  an  attack  of  colic. 
It  usually  attacks  the  upper  extremities,  by  preference,  although  there 
is  always  some  slight  involvement  of  the  lower  limbs.  Definite  lower 
limb  palsies  are  rare,  and  usually  occur  only  in  children.  The  pareses 
predominate  particularly  in  the  extensors  of  the  index  finger  and  thumb 


284  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

— the  sensory  symptoms,  hyperesthesiee,  pains,  nerve  tenderness, 
Lasegue's  phenomenon,  paresthesise,  are  usually  much  less  than  in 
alcoholic  neuritis.  The  supinator  longus  is  very  frequently  spared. 
The  paralyses  are  usually  symmetrical,  but  may  be  quite  irregular; 
the  proximal  trunk  muscles  may  be  involved^ — the  distal  ones  free. 
This  occasionally  happens  in  alcoholic  neuritis  as  well.  Reactions 
of  degeneration  appear  in  the  paretic  muscles.  Anesthesia,  atrophies, 
trophic  disturbances,  and  contractures  are  met  with,  but  may  be 
considered  exceptional.  Oculomotor  palsies  also  occur,  and  optic 
nerve  atrophy  is  not  infrequent.  Other  cranial  nerves,  those  of  the 
larynx,  pharynx,  and  face  are  also  implicated,  though  rarely.  Lead 
encephalopathies  resembling  those  of  alcohol  are  known. 

Course  and  Treatment. — The  course  of  lead  polyneuritis  is  essentially 
chronic,  lasting  from  several  months  to  a  year.  The  prognosis  is 
usually  favorable.  The  treatment  is  the  same  as  for  neuritis  in 
general,  with  the  addition  of  excessive  diuresis,  and  the  use  of  such 
remedies  that  may  hasten  lead  elimination,  such  as  potassium  iodid. 

Arsenical  Neuritis. — Dejerine,^  in  1883,  first  insisted  on  the  essential 
similarities  of  neuritis  of  alcoholic  and  arsenical  origin,  which  view- 
point has  been  amply  verified  in  the  extensive  studies  following  a 
severe  epidemic  of  arsenical  polyneuritis  in  England,  in  1899-1900.- 
The  most  frequent  source  of  arsenical  poisoning  has  been  shown  to 
come  from  impure  glucose  products;  the  sulphuric  acid  used  to  con- 
vert the  starch  containing  arsenic.  Other  sources  are  wall-papers, 
certain  manufactures  (dye-stuffs),  artificial  flowers,  cosmetics,  beauty 
pastes  and  powders,  hair  dyes,  and  arsenic  used  in  medicines.  The 
pathological  alterations  (a  parenchymatous  neuritis)  do  not  difi^er 
from  those  seen  in  alcoholic  neuritis. 

Symptoms. — ^Acute  polyneuritis  from  arsenical  poisoning  is  rare.  It 
sets  in  shortly  after  the  gastro-intestinal  symptoms  of  acute  toxemia 
have  passed. 

In  the  chronic  cases  the  general  symptoms  of  chronic  arsenic  intoxi- 
cation are  first  observed.  These  are  the  anorexias,  congestions  of 
the  upper  respiratory  tract  (nasal  catarrh,  cough)  or  more  frank 
diarrheas  of  gastro-intestinal  irritation.  The  neuritis  develops  simul- 
taneously with  the  symptoms  of  chronic  intoxication. 

As  with  alcoholic  neuritis,  sensory  symptoms,  paresthesise,  hyperes- 
thesise,  numbness,  shooting  pains,  sweating,  develop  first.  A  pig- 
mented condition  of  the  skin,  most  marked  about  normally  pigmented 
areas,  is  found  in  the  majority  of  the  cases.  The  pigmentation  may 
become  very  general  and  very  dark,  and  is  associated  with  herpetic, 
eczematous,  or  scaly,  papular  eruptions.  Certain  of  the  newer  pre- 
parations of  arsenic  which  have  been  extensively  advocated  for  the 
treatment  of  syphilis,  notably  atoxyl  and  arsacetin,  are  reported  to 
have  caused  optic  nerve  atrophy  with  blindness. 

'  Comptes  Rendus,  October,  1883,  vol.  xcviii,  No.  17. 
2  Lancet,  1900,  vol.  i,  p.  1610. 


INFECTIOUS  NEURITIS  285 

The  signs  of  sore  nerve  trunks,  Lasegue's  phenomenon,  loss  of  motor 
power  in  both  extremities,  are  present  in  arsenical  cases.  Ataxias 
occur,  and  cases  of  arsenical  neuritis  have  been  confounded  with  tabes. 

The  prognosis  is  usually  good,  but  the  blindness  has  been  permanent. 

Other  Intoxications. — Carbon  monoxide,  and  illuminating  gas 
poisoning,  if  severe  and  not  lethal,  frequently  develop  a  severe  grade 
of  multiple  neuritis  not  differing  in  any  marked  degree  from  alcoholic 
polyneuritis.  The  gases  found  in  natural  gas,  and  in  many  artificial 
gases  contain  the  same  chemical  radicals  as  alcohol,  and  the  toxic 
action  is  identical.  In  the  very  severe  cases,  polioencephalitis  develops 
with  multiple  softenings,  not  entirely  confined  to  the  thalamus  or 
corpora  striata. 

Carbon  bisulphide,  which  is  extensively  used  in  rubber  industries, 
may  give  rise  to  a  multiple  neuritis.  The  toxic  ion  is  not  definitely 
known.  Similar  poisoning  results  from  sulphonal  and  trional,  two 
sulphuric  acid-alcohol  hypnotics.  A  number  of  the  nitrobenzol  series 
can  produce  localized  or  general  neuritis. 

Phosphorus,  mercury,  copper,  and  silver  can  produce  poisoning  with 
the  development  of  multiple  neuritis. 

Infectious  Disease  Types. — Mild  or  severe  general  neuritis  has  been 
observed  to  have  occasionally  followed  practically  every  known 
infectious  disease. 

Diphtheria. — Here  the  neuritis  is  rarely  generalized,  and  the  cranial 
nerves  bear  the  chief  brunt  of  the  poison.  In  mild  forms  the  soft 
palate  and  pharyngeal  muscles  are  first  or  alone  implicated.  Oculo- 
motor palsies  are  also  frequent.  Some  degree  of  facial  palsy  is  also 
met  with.  In  the  severe  types  the  larynx,  tongue  and  the  pneumo- 
gastric  innervations  become  invaded. 

Generalized  forms  occur,  differing  in  no  essential  particular  from  the 
types  already  described. 

Diphtheritic  palsies  may  come  on  soon  in  the  disease  or  they  may 
follow  a  month  or  six  weeks  after  the  subsidence  of  the  disease  proper. 
For  those  patients  who  develop  polyneuritis  the  diagnosis  is  usually 
grave.  The  cranial  nerve  types  are  usually  less  severe,  although 
occasionally  one  finds  pneumogastric  palsies  which  are  fatal. 

Influenza. — The  toxins  of  the  influenza  organism  seem  to  have  a 
special  attraction  for  sensory  nerve  structures.  Neuralgias,  localized 
neuritides,  are  extremely  common,  and  polyneuritis  not  a  rarity.  The 
polyneuritis  is  of  a  parenchymatous  type,  is  usually  mild,  quite  irregu- 
lar, and  differs  in  no  essential  manner  from  other  types  described. 
Its  course  is  rarely  over  a  few  months  and  the  prognosis  is  usually 
good. 

Polyneuritis  of  a  mild  parenchymatous  type  occurs  as  a  sequel  of 
typhoid  fever,  smallpox,  erysipelas,  pneumonia,  pleurisy,  acute  articu- 
lar rheumatism,  parotitis,  gonorrhea,  dysentery,  measles,  Pasteur 
rabies  treatment,  whooping  cough,  and  peripheral  septicemia. 

In  chronic  tuberculosis  mild  grades  of  neuritis  are  frequent,  and 


286 


AFFECTIONS  OF   THE  PERIPHERAL  NERVES 


severe  polyneuritis  is  occasionally  met  with  in  the  marantic  type. 
Syphilis  rarely  causes  a  polyneuritis,  but  it  is  known.  Malaria  is 
also  a  rare  cause.     Leprosy  causes  a  specialized  form. 

PLEXUS  PALSIES. 

Plexus  or  root  palsies  are  comparatively  rare.  They  occur  more 
often  in  the  upper  extremity.  Brachial  plexus  palsy,  as  Erb's  birth 
palsy,  is  the  type.  Lumbar  plexus  palsies  rarely  occur  alone  uncom- 
plicated by  cord  lesions,  since  they  are  usually  produced  by  compres- 
sion, either  resulting  from  tumor,  fracture.  Pott's  disease,  etc.  Sacral 
plexus  palsies,  however,  are  not  infrequent.  They  make  up"  the 
classical  cauda  equina  lesions,  arising  from  the  pressure  of  a  tumor, 


Fig.   136. 


-Sensory  disturbances  in  seven  cases  of  gunshot  wounds  of  the   brachial 
plexus.     (Edinger.) 


from  hemorrhage,  fracture  of  the  sacrum,  bony  disease  of  the  lumbar 
vertebrae,  pelvic  tumor,  abscess,  etc. 

Brachial  Plexus  Palsies. — These  most  frequently  arise  from  pulling 
or  wrenching  of  the  arms  from  accidents.  Dislocation  of  the  shoulder- 
joint  can  cause  a  plexus  injury.  Cervical  rib  is  a  rare  cause,  aneurism 
of  the  subclavian  also.  Gunshot  wounds  occasion  them.  (See  Fig. 
136.) 

The  plexus  is  made  up  of  the  lower  four  cervical  and  the  upper  dorsal 
root.  Extraspinal  as  well  as  intraspinal  lesions  go  to  make  up  the 
symptom  picture.  This  picture  is  rarely  complete,  but  as  the  fibers 
making  up  the  plexus  have  a  fairly  definite  arrangement  so  far  as 
muscular  distribution  is  concerned,  a  study  of  the  muscles  involved 
gives  a  key  to  the  roots  injured. 

The  fifth  and  sixth  cervical  roots  contain  the  fibers  going  to  the 


PLEXUS  PALSIES 


287 


deltoid,  biceps,  brachialis  anticus,  supinator  longus,  supra-  and  sub- 
scapularis,  the  clavicular  fascia  of  the  pectoralis  major,  and  the 
serratus  magnus.  The  seventh  cervical  root  contains  the  fibers  dis- 
tributed to  the  triceps,  the  sternal  portion  of  the  pectoralis  major, 
to  the  dorsalis  magnus,  to  the  extension  of  the  wrist,  and  also  some 
filaments  to  the  median  and  ulnar  nerves. 

The  eighth  cervical  and  the  eighth  dorsal  form  the  brachial,  internal 
cutaneous,  ulnar,  median  and  parts  of  the  radial  with  their  muscular 
innervations,  as  seen  in  the  illustrations.  (See  Figs.  11  to  16,  also 
Fig.  126.) 


Figs.  137,  138,  and  139. — Distribution  of  radicular  sensory  disturbances  in  case  of 
traumatic  brachial  palsy  of  the  left  side.  There  was  total  paralysis,  atrophy,  and  loss  of 
tendon  reflexes,  edema,  and  cyanosis  of  left  arm.  Abolition  of  all  types  of  sensibility 
of  the  distribution  of  the  Cv  to  Di.  Myosis  and  enophthalmus  in  left  eye.  (Dejerine- 
Klumpke.) 


The  clinical  pictures  seen  are  those  of  a  total  brachial  plexus  palsy; 
a  superior  and  inferior  type. 

Total  Brachial  Palsy. — Here  all  of  the  muscles  of  the  hand  are  par- 
alyzed, those  of  the  forearm,  the  arm  and  the  shoulder.  The  arm 
hangs  limp  like  a  flail.  In  the  early  stages  the  skin  is  cyanosed, 
there  are  severe  pains  (in  the  accidental  cases),  and  a  loss  of  the  secre- 
tion of  sweat.  Atrophy  comes  on  quickly  and  is  extreme,  with  loss 
of  electrical  responses.    Trophic  disturbances  are  usually  present. 


AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

Sensory  disturbances  are  present.  There  is  loss  to  all  forms  of 
sensibility,  including  the  sense  of  position  in  the  hands  and  the  fore- 
arm anteriorly  and  posteriorly.  Anteriorly  the  upper  border  of  anes- 
thesia ceases  just  above  the  internal  condyle;  externally  it  extends  to 
the  insertion  of  the  deltoid. 

Furthermore,  since  cervical  sympathetic  fibers  are  represented 
in  the  communicating  branch  of  the  first  dorsal,  one  finds  oculopupil- 
lary  signs  first  demonstrated  by  Madame  Dejerine-Klumpke  (1885). 
They  consist  of  a  contraction  of  the  pupil  on  the  paralyzed  side,  a 
diminution  in  the  size  of  the  palpebral  fissure  (pseudoptosis)  and  a 
retraction  of  the  globe  of  the  eye  within  the  orbit  (enophthalmus) . 

Furthermore,  lesions  here  frequently  cause  changes  in  the  sweat 
secretion.     These  are  sharply  delimited  to  the  head,  neck,  face,  to  the 


Fig.  140. — Erb's  birth  palsy.     (Frauenthal.) 

level  of  the  third  rib  in  front  and  the  spine  of  the  scapula  behind, 
including  the  upper  extremity,  all  confined  to  the  side  injured. 

Inferior  Root  Type. — ^This  has  also  been  designated  the  Klumpke 
palsy.  It  follows  a  less  complete  lesion  of  the  plexus,  involving  the 
eighth  cervical  and  the  first  dorsal.  It  may  result  from  injury,  birth 
palsy  (Erb's  palsy),  from  cervical  rib,  or  from  syphilitic  deposits. 
The  small  muscles  of  the  hand  are  involved.  The  atrophy  is  rapid. 
There  is  edema  of  the  skin,  cyanosis,  perhaps  trophic  changes  in  the 
nails.  The  anesthesia  is  less  extensive,  being  limited  to  the  distribution 
of  the  radial  and  internal  cutaneous  nerves.  The  oculopupillary 
signs  are  evident. 

Superior  Brachial  Plexus  Palsy. — Here  the  upper  branches,  fifth  and 
sixth  cervical,  are  involved.  Duchenne  described  the  earlier  cases 
minutely.    The  palsied  muscles  have  been  mentioned.    The  sensory 


PLEXUS  PALSIES 


289 


disturbances  are  more  extensive  than  those  observed  in  the  inferior 
type.    The  pupillary  symptoms  are  absent. 

Mixed  Types. — These  are  more  frequent.  The  more  common  form 
of  so-called  Erb's  palsies  belong  here.  Many  of  these  are  bilateral, 
the  two  arms,  however,  being  dissimilarly  involved.  The  study  of  the 
muscles  involved  in  the  atrophy,  the  electrical  changes,  the  sensory 
changes,  to  light,  touch,  heat,  cold,  pain,  bony  sensibility,  and  to 
position,  the  presence  of  trophic  disturbances  (protopathic  system 
changes),  and  the  oculopupillary  phenomena  determine  the  roots 
involved.     (See  Figs.  11  to  16.) 

There  are  a  number  of  bony  conditions  which  can  determine  brachial 
plexus  palsies.     Tuberculous,  syphilitic,  carcinomatous,  sarcomatous, 


Cvn 


C  VI.  vn 


Figs.  141  and  142. — Topography  of  tactile,  pain,  and  thermal  sensory  disturbances  in 
a  superior  brachial  palsy  type  due  to  injury  to  the  shoulder.  The  fifth,  sixth,  and 
seventh  cervical  roots  are  involved.    (Dejerine.) 


arthritic  infiltrations  about  the  vertebral  canal  impinging  upon  the 
cords  of  the  plexus  can  give  rise  to  palsies  of  this  type.  Similar 
changes  not  infrequently  also  cause  pictures  which  are  often  confused 
with  brachial  neuritis.  Some  of  the  severe  arm  pains  with  mild  atro- 
phies are  forms  of  brachial  radiculitis  (q.  v.),  either  inflammatory  or  trau- 
matic in  origin.  In  the  course  of  a  rheumatoid  arthritis  one  not 
infrequently  encounters  these  radicular  disturbances  which  are 
undoubtedly  referable  to  a  vertebral  arthritis. 

Course. — General  statements  regarding  the  course  are  misleading. 

The  majority  of  Erb's  palsies  due  to  obstetrical  accidents  recover, 

especially  when  only  a  few  roots  of  the  plexus  are  involved.     Total 

separation  from  the  cord,  as  in  severe  dislocations,  falls,  etc.;  with 

19 


290 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


complete  plexus  palsy,  usually  means  an  incurable  affection,  not 
remediable  by  surgery. 

The  underlying  etiological  factor  determines  the  course  in  a  number 
of  others.  Palsies  caused  by  cervical  rib,  or  subclavian  aneurism  do 
not  get  well  spontaneously,  nor  does  iodide  help  them.  Proper  surgery, 
as  indicated  by  the  a^-ray  findings,  may  be  of  service. 

Diagnosis. — The  rarer  nuclear  and  neuritic  atrophies  and  dystrophies 
have  been  mistaken  for  these  radicular  palsies,  but  the  study  of  the 
sensory  changes  should  at  once  establish  the  differences.  Spinal 
gliosis  of  the  upper  arm  type  will  show  dissociation  symptoms,  indicat- 
ing the  intramedullary  nature  of  the  lesion.    Such  a  dissociation  is  a 


Fi(S.   143. — Double  cervical  rib,   causing  mixed  type  of  brachial  palsy.     (Goodhart.) 


retention  of  tactile  sensibility  with  loss  of  pain  and  thermal  sensi- 
bility. Various  neuritides,  lead,  diabetes,  have  a  peripheral  rather 
than  a  root  distribution.  Certain  exceptional  cases  of  tabes,  with 
atrophy,  can  be  separated,  but  may  require  a  cerebrospinal  fluid 
examination.  Syphilitic  cervical  spondylitis  cases  at  times  show  these 
symptoms. 

Treatment. — There  is  little  treatment  for  the  severe,  tearing  lesions 
of  the  plexus.  Whether  they  can  be  looped  up  with  other  roots  to 
advantage  has  to  be  answered  in  the  future. 

The  cause  of  the  palsies,  other  than  tearing,  can  often  be  removed. 
Thus  surgical  treatment  of  neck  glands,  subclavian  aneurisms,  cervical 


PLEXUS  PALSIES  291 

rib,  spondylitis,  and  osteo-arthritis  is  efficacious.  The  treatment  of  a 
cervicodorsal  Pott's  by  proper  fixation,  and  the  antisyphilitic  treat- 
ment of  a  luetic  spondylitis  are  satisfactory.  Rare  cases,  often  bilat- 
eral, due  to  profound  secondary  anemia,  are  often  very  refractory. 
True  radicular  neuritis  is  also  stubborn. 

The  pains  are  best  relieved  by  analgesics;  antipyrin,  aspirin,. pyram- 
idon,  or  other  combinations  are  valuable.  Heat  is  usually  harmful, 
and  massage  contra-indicated  in  the  early  stages.  Osteopathic  man- 
ipulation is  a  dangerous  procedure  in  the  early  stages.  In  certain 
osteo-arthritic  cases  it  proves  valuable  later. 

Simple  counter-irritation  over  the  site  of  the  plexus,  above  the 
clavicle,  is  invaluable  in  many  mild  neuritic  attacks;  while  for  the 
severer  attacks  high-frequency  currents,  violet  light  therapy,  Leduc 
current  at  times  cause  great  relief. 

Dietetic  and  general  management  in  the  neuritic  cases  is  not  to  be 
overlooked.  They  need  fats  in  ample  proportion.  This  is  best  supplied 
through  taking  large  quantities  of  milk. 

Lumbosacral  Plexus. — The  attention  of  the  neurologist  is  often 
focussed  about  the  process  of  delivery.  Whereas  it  is  the  child  that 
occasionally  has  a  birth  palsy  which  is  brachial,  it  is  the  mother  who 
has  a  lumbosacral  palsy  due  to  long-continued  pressure  of  the  head 
upon  the  plexus.  Here  the  palsy  may  be  partial,  or  it  may  be  fully 
developed,  resembling  a  palsy  due  to  a  lesion  of  the  cauda  equina. 

Intra-abdominal  pressure  may  also  arise  from  bony  tumors,  from 
pelvic  inflammations,  and  pus  collections  of  the  pelvis  due  to  old 
appendicitis,  salpingitis,  etc.  Gunshot  wounds  occasion  sacral  plexus 
palsies. 

Intramedullary  causes  for  lumbosacral  plexus  palsies  are  more  fre- 
quent than  for  brachial  palsies,  because  of  the  arrangement  of  the 
nerves. 

The  Cauda  equina  comprises  the  entire  group  of  coccygeal,  sacral 
and  three  last  lumbar  roots.  The  second  lumbar  is  practically  included 
within  the  canal.     (See  Figs.  15  and  16.) 

Symptoms. — In  the  fully  developed  picture  one  finds  complete 
flaccid  palsy  of  the  lower  extremities.  There  is  marked  toe-drop, 
and  limitation  of  all  of  the  motions  of  the  legs.  Atrophy  of  the 
muscles  takes  place  rapidly,  especially  of  the  lower  extremities.  The 
muscles  of  the  anterior  portion  of  the  thigh  innervated  in  part  by  the 
second  lumbar,  remain  normal  and  active.  Fibrillary  twitchings  are 
frequent  in  the  atrophied  muscles,  and  reaction  of  degeneration  is  present. 

The  cutaneous  reflexes  are  usually  absent,  the  Achilles  reflex  is 
absent  and  that  of  the  patellar  as  weH  unless  the  lesion  strikes  below 
the  third  lumbar  root. 

Pains  are  usuaUy  very  marked  and  persistent.  They  occur  in 
paroxysms,  and  are  usuafly  extreme.  The  chief  path  is  that  of  the 
sciatic,  but  they  may  be  located  almost  anywhere  about  the  thigh. 
They  may  be  bilateral  or  unilateral  and  shift  considerably. 


292 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


Sensory  examination  shows  typical  diminution  of  all  forms  of 
sensibility  following  the  neuritic  type.  Lesions  higher  up  involving 
the  con  us  or  the  cord  show  dissociated  sensory  phenomena  to  be 
discussed  later. 

The  touch  anesthesia  extends  to  the  limit  of  the  second  dorsal, 
i.  e.,  at  the  upper  level  of  the  sacrum,  including  the  anus,  perineum, 
and  genito-urinary  organs.     (See  Plates  X  and  XL) 

Vasomotor  phenomena,  cold,  somewhat  cyanotic  skin,  and  hand 
edema  may  be  present.  The  bladder,  rectum,  and  genital  controls 
are  involved.  This  is  the  usual  picture  in  a  total  lesion.  In  practice 
this  is  less  often  seen  than  the  many  irregular  types. 

Course. — Prognosis, — Limiting  the  discussion  to  the  cases  of  pressure 
of  parturition  one  finds  that  these  patients  often  suffer  from  irregular 
sciatic  pains  in  the  later  stages  of  their  pregnancy.  A  few  days  after 
a  protracted  delivery  the  pains  often  increase  very  markedly  and  then 
a  paralysis  of  the  lower  extremities  develops.    This  is  usually  more 


Fig.  144. — Sensory  disturbances  in  five  cases  of  sacral  plexus  injury  due  to  gunshot 
wounds.  In  the  first  case  there  was  a  total  palsy  in  the  peroneus  and  tibialis  nerve 
distribution;  in  the  second  the  peroneus  alone  was  involved;  in  the  third  the  peroneus, 
tibialis,  ilio-inguinalis  and  obturator;  in  the  fourth  the  peroneus  and  tibialis;  in  the 
fifth  the  crural  nerve  distribution  alone  suffered.     (Edinger.) 

marked  on  one  side.  Bladder  and  rectal  difficulties  arise  and  the 
patient  shows  the  picture  usually  of  an  incomplete  plexus  palsy  of  the 
lumbosacral  region.  After  a  month  or  so — sometimes  several  months 
in  the  severe  cases — a  complete  recovery  usually  takes  place. 

The  results  of  sacral  plexus  injury  by  gunshot  wounds  are  less 
promising. 

Treatment. — Is  that  for  a  neuritis  in  general  (g.  v.) . 


PERIPHERAL  PALSIES. 

Numerous  forms  of  peripheral  nerve  palsy  are  found,  varying  accord- 
ing to  the  particular  fibers  involved.  The  branches  that  arise  from 
the  brachial  plexus  have  already  been  described.  Any  of  these  may 
be  injured  or  inflamed  with  a  resulting  partial  or  complete  palsy.  The 
various  neuritides  will  not  be  reexamined  and  the  following  sections 
will  deal  with  those  peripheral  palsies  largely  due  to  defect  or  injury. 


PERIPHERAL  PALSIES  293 

Defects,  Congenital  and  Acquired. — ^Muscular  defects  in  the  upper 
and  lower  extremities  are  by  no  means  uncommon.  They  have 
been  described  for  some  centuries.  Their  precise  formulation  began 
with  Ziemnsen's  work  in  1857.  The  later  literature  is  summed  up  by 
Bing/  Lorenz,2  and  by  Hirschfeld.^  The  causes  for  these  muscular 
defects  are  extremely  complicated. 

The  occurrence  is  very  manifold.  Bing  was  the  first  to  collect  the 
entire  group,  and  Abromeit,  in  1909,  completed  the  study  which  shows 
that  an  absence  of  any  muscle  of  the  body  may  be  expected.  One  in 
10,000  shows  such  defects. 

In  the  majority  of  the  cases  the  defect  is  unilateral,  and  they  are 
more  frequent  in  men. 

Abromeit  collected  reports  of  186  cases  of  defect  of  the  pectoral 
muscles,  the  sternocostal  portion  being  most  affected.*  This  seems 
the  most  frequent  of  such  anomalies.  The  trapezius  was  absent 
in  33  cases,  the  serratus  magnus  in  22,  quadratus  26,  omohyoid  16, 
semimembranosis  7,  rectus  muscles  11,  pyramidalis,  often  found 
absent  postmortem,  diaphragm  3  times,  deltoid  5,  sternocleidomastoid 
8,  etc.     The  smaller  muscles  of  the  hand  are  not  infrequently  absent. 

Certain  combinations  of  muscles  may  be  absent,  constituting  a 
group  complex.  Atrophy  or  loss  of  other  parts  may  be  combined 
and  gross  anomalies  of  structure  may  be  combined  with  marked 
muscular  defects;  various  monsters,  syndactylia,  etc.  The  hereditary 
nature  of  syndactylous  anomalies  has  given  occasion  for  some  important 
studies. 

The  symptoms  involve  the  physiological  loss  of  the  special  muscle 
function  or  the  resultant  of  function  from  the  muscles  present  in  a 
combination. 

These  patients  rarely  show  a  marked  loss  of  eJEciency,  especially 
with  one-sided  defect,  as  do  those  who  acquire  a  defect.  Acquired 
defects  are  usually  bilateral.  They  usually  involve  a  group  of  muscles; 
the  disease  rarely  causes  a  total  loss  of  muscle  substance,  and  anomalies 
of  accompanying  structures  are  missing. 

Fibrillary  twitchings  are  often  diagnostic  of  the  acquired  muscular 
defects. 

The  prognosis  and  treatment  require  little  discussion.  Gymnastic 
exercise  of  the  residual  muscular  combinations  directed  to  the  acquisi- 
tion of  greater  efficiency  by  skilful  application  of  mechanical  principles 
is  always  an  individual  goal  that  cannot  be  more  than  mentioned  here. 

Peripheral  Palsies  Due  to  Nerve  Injuries. — This  section  discusses 
peripheral  palsies  due  to  nerve  injuries  rather  than  to  those  due  to 
general  neuritic  processes.  An  injury  may  affect  a  nerve  either  in  its 
continuity,  or  in  one  of  its  terminal  branches,  thus  giving  rise  to 
different  symptoms,  and  requiring  a  diverse  therapy. 

1  Virch.  Arch.,  170,  1902.  ^  Die  Muskel  Erkrankungen  Nothnagel,  1904. 

^  Kongenitale  Muskeldefekte,  Lewandowsky  Handbuch,  1911. 
^  See  Lewandowsky,  II,  p.  253. 


294  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

Injury  in  continuity  may  result  from  penetrating  wounds,  traction, 
pressure,  blows  or  by  operation.  They  may  give  rise  to  complete  or 
incomplete  division. 

Symptoms. — In  complete  division  Sherren  and  Head  have  shown 
that  the  afferent  peripheral  fibers  may  be  divided  into  three  systems: 

1.  Those  which  subserve  deep  sensibility  and  conduct  the  impulses 
produced  by  pressure.  The  fibers  of  this  system  run  mainly  with 
the  motor  nerves  and  are  not  destroyed  by  division  of  all  the  sensory 
nerves  of  the  skin. 

In  a  part  innervated  only  by  this  system  gradual  increase  in  press- 
ure can  be  recognized  and  pain  experienced  when  such  pressure 
becomes  excessive.  The  patient  can  also  appreciate  the  extent  and 
direction  of  movements  produced  passively  in  all  the  joints  within 
the  affected  area. 

2.  Those  which  subserve  protopathic  sensibility.  This  system  of 
fibers  and  end-organs  respond  to  painful  cutaneous  stimuli  and  to 
the  extremes  of  heat  and  cold;  it  also  endows  the  hairs  with  power  to 
react  to  painful  stimuli.  The  distribution  of  the  protopathic  fibers 
usually  overlaps  greatly  the  area  supplied  by  similar  fibers  from 
adjacent  nerves. 

3.  Those  which  subserve  epicritic  sensibility.  The  nerve  fibers 
and  end-organs  of  this  system  endow  the  part  with  the  power  of 
responding  to  light  touch  with  a  well-localized  sensation.  The  exist- 
ence of  this  system  enables  one  to  discriminate  two  points  and  to  appre- 
ciate the  difference  between  cold  and  heat.  The  distribution  of  these 
fibers  in  large  peripheral  nerves,  such  as  the  median  and  ulnar,  has 
very  little  overlap  compared  with  the  greater  overlapping  of  the 
protopathic  supply. 

These  investigations  were  carried  further,  particularly  with  regard 
to  deep  sensibility  and  the  distribution  of  heat  and  cold  spots  by 
Head  and  Rivers  after  voluntary  section  of  the  radial  and  external 
cutaneous  nerves  in  the  former's  arm. 

To  illustrate  these  changes  in  sensibility  after  division  of  a  mixed 
nerve  the  ulnar  is  an  excellent  example  (Fig.  149).  After  complete 
division  of  this  nerve  at  the  wrist,  if  no  tendons  have  been  divided 
at  the  same  time,  the  patient  is  able  to  appreciate  those  stimuli  com- 
monly called  tactile.  A  touch  with  anything  which  deforms  the  skin 
may  be  readily  appreciated  and  correctly  localized.  When  pricked 
with  a  pin  the  patient  knows  that  he  has  been  touched  but  fails  to 
perceive  the  sharpness  of  the  stimulus  (deep  sensibility).  But  if  ten- 
dons are  divided  at  the  same  time  or  the  section  involves  the  nerve 
above  the  point  at  which  its  muscular  branches  are  given  off,  deep 
touch  may  be  unperceived.  These  characteristics  are  of  the  utmost 
importance,  many  cases  of  nerve  injury  have  been  overlooked  from 
failure  to  recognize  these  facts. 

The  point  of  a  pin  and  all  temperatures  are  unappreciated  within 
an   area   which   varies   somewhat  in  each  case  (loss   of  protopathic 


PERIPHERAL  PALSIES 


295 


sensibility)-  Surrounding  this  area  and  corresponding  closely  to 
the  distribution  of  the  nerve  as  figured  in  Plates  X  and  XI  is  a 
territory  within  which  the  patient  is  unable  to  appreciate  light 
touches  with  cotton-wool  and  temperatures  between  about  22°  and 
40°  C.  (minor  degrees  of  temperature),  and  fails  to  discriminate  the 
points  of  a  pair  of  compasses  when  separated  to  many  times  the 
distance  necessary  over  the  corresponding  portion  of  the  sound  limb 
or  the  unaft'ected  portion  of  the  injured  one  (loss  of  epicritic  sensibility). 
Within  this  area  of  loss  of  sensibility  to  light  touch,  to  which  we  gave 
the  name  of  "intermediate  zone"  the  patient  is  able  to  appreciate  the 
sharpness  of  a  pin-prick  and  to  differentiate  temperatures  below  20°  C. 
and  above  45°  C,  naming  them  correctly. 


Fig.  145. — To  illustrate  the  changes  in  sensibility  met  with  after  complete  division 
of  a  peripheral  nerve.  The  area  inclosed  by  a  line  is  that  in  which  epicritic  sensibility 
is  lost.  The  shaded  area  is  that  of  loss  of  epicritic  and  protopathic  sensation.  The 
unshaded  portion  is  the  "intermediate  zone."      (Sherren.) 


Division  of  a  peripheral  nerve  produces  a  well-defined  loss  of  epicritic 
sensibility,  a  smaller  loss  of  protopathic  sensibility  with,  as  a  rule,  ill- 
defined  limits.    In  many  cases  there  is  no  loss  of  deep  sensibility. 

Complete  division  of  certain  nerve  branches  produces  no  objective 
change  in  sensibility,  these  are  the  musculospiral  below  the  point  at 
which  its  external  cutaneous  branches  are  given  oft',  the  radial,  and 
certain  cervical  anterior  primary  divisions. 

Symptoms  in  Incomplete  Division. — The  sensory  sj^mptoms  may  be 
nil.  The  patient  is  conscious  of  an  area  altered  in  sensibility^,  and  it  is 
often  possible  to  demonstrate  this  by  the  changed  sensibility  produced 
at  its  borders  when  a  piece  of  cotton-wool  or  the  point  of  a  needle  is 
dragged  lightly  across  the  skin  from  sound  to  affected  portions  (line 


296 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


of  change).  If  the  area  of  changed  sensibiHty  is  well  marked,  response 
to  the  compass  test  will  be  defective. 

In  cases  of  greater  severity  the  loss  of  sensibility  to  cotton-wool  may 
be  absolute,  with  borders  as  well  defined  as  after  complete  division. 

When  the  injury  is  more  severe,  impairment  or  loss  of  protopathic 
sensibility  results  and  the  sensory  loss  may  resemble  exactly  that  seen 
after  complete  division. 

Sherren  has  shown  that,  contrary  to  the  usual  teaching,  the  motor 
loss  in  incomplete  division  is  not  more  than  the  sensory  loss.  Paralysis 
of  muscles  of  the  injured  nerve  distribution  may  result  after  eight  to 
fourteen  days.  The  usual  reaction  on  or  about  the  tenth  day  is  that 
the  muscles  do  not  respond  to  the  interrupted  current  but  do  react 


Fig.  146. — Loss  of  sensibility  after  complete  division  of  ulnar  nerve.     (Sherren.) 


to  the  constant  current.  The  strength  of  the  current  needed  to  cause 
a  contraction  is  less  on  the  sound  side;  the  contraction  is  brisk  in 
comparison  to  the  R.  D.  and  polar  reversal  is  absent. 

Pain  is  a  frequent  after-result  in  incomplete  division;  there  may  also 
be  tender  and  glossy  skin  and  changes  in  the  nails. 

Stages  of  Recovery. — Following  complete  division,  in  from  six  to 
sixteen  weeks,  the  restoration  of  protopathic  sensibility  commences 
and  is  completed  in  from  four  to  twelve  months  after  suture  of  divided 
nerves.  Blisters  may  occur  early,  but  later  all  ulcers  heal;  blisters 
no  longer  appear.  Epicritic  touch  is  unchanged  throughout,  but  in 
from  twelve  to  fifteen  months  the  whole  area  is  sensitive  to  light  touch 
and  intermediate  degrees  of  temperature.  Improvement  in  the  power 
of  accurate  localization  is  the  third  stage,  and  is  tested  out  by  the  com- 


PERIPHERAL  PALSIES 


297 


pass.    The  motor  recovery  is  gradual,  the  electrical  reactions  of  incom- 
plete division  first  appearing. 


Fig.  147. — End  of  first  stage  of  recovery.    (Sherren.) 


Fig.  148. — Commencement  of  second  stage  of  recovery, 
area  regaining  sensibility  to  cotton-wool. 


The  dotted  line  marks  the 
(Sherren.) 


It  seems  certain  that  no  regeneration  takes  place  in  the  peripheral 

end  of  a  divided  nerve  without  union  with  the  central  nervous  system. 

After  incomplete  division  of  a  mixed  nerve  the  loss  of  sensation  and 


298 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


motion  may  at  first  resemble  that  which  follows  complete  division, 
but  the  method  of  recovery  is  entirely  different. 


Fig.  149. — Loss  of  sensibility  after  complete  division  of  ulnar  nerve. 


Fig.  150. — Showing  method  of  recovery  after  incomplete  division.     (Sherren.) 


After  complete  division  of  a  nerve  and  suture,  sensibility  to  prick  is 
restored  before  the  commencement  of  recovery  of  sensibility  to  light 
touch.     Complete  sensory  recovery  often  occupies  several  years. 


PERIPHERAL  PALSIES  299 

But  after  incomplete  division  sensibility  to  light  touch  and  to  prick 
are  restored  together  and,  unless  nerve  fibers  have  been  anatomically 
divided  in  considerable  number,  the  power  of  appreciating  two  points 
(the  compass  test)  is  soon  regained.  This  is  an  extremely  important 
point,  for  upon  the  recovery  of  this  power  of  localizing  depends  the 
utility  of  the  part  for  fine  work.  It  is  important  to  recognize  that  in 
injuries  of  nerves  without  interruption  of  their  anatomical  continuity 
the  power  of  localization  returns  quicljly,  unless  the  injury  has  been 
sufficient  to  cause  complete  division;  in  this  case  the  usual  three  stages 
are  present,  but  the  time  of  the  third  stage  is  much  shortened. 

Knowledge  of  this  method  of  sensory  recovery,  first  described  by 
Head  and  Sherren,  is  a  valuable  addition  to  our  powers  of  diagnosis. 
If  both  forms  of  sensibility  are  recovering  together,  it  is  certain  that 
the  injury  has  not  been  severe  enough  to  produce  complete  interrup- 
tion of  conduction  in  the  injured  nerve,  with  degeneration  of  the  whole 
peripheral  end. 

Motor  recovery  after  incomplete  division  follows  the  same  march 
as  after  complete  division;  the  muscles  nearest  the  seat  of  the 
injury  first  regain  voluntary  power  and  excitability  to  the  interrupted 
current. 

In  the  cases  in  which  the  reactions  typical  of  incomplete  division 
are  present,  voluntary  power  usually  returns  before  the  reestablish- 
ment  of  excitability  to  the  interrupted  current. 

Sensory  recovery  usually  begins  in  about  three  weeks,  and  is  com- 
plete in  about  six  months.  Motor  recovery  in  from  a  few  days  to  ten 
weeks. 

These  times  of  motor  and  sensory  recovery  are  approximate  only, 
and  vary  with  the  severity  of  the  injury  and  its  distance  from  the 
periphery.  When  epicritic  sensibility  alone  is  lost  recovery  is  much 
more  rapid  than  when  both  forms  of  sensibility  are  affected.  When 
the  injury  affects  the  brachial  plexus,  considerably  longer  time  is 
necessary  for  the  commencement  and  progress  of  recovery. 

To  sum  up,  after  incomplete  division  of  a  mixed  nerve,  both  forms 
of  sensibility  (epicritic  and  protopathic),  if  lost,  return  at  the  same 
time,  commencing  at*  a  date  which  varies  with  the  distance  of  the 
injury  from  the  periphery  from  about  three  weeks  at  the  wrist  to  six 
months  in  the  plexus,  and  also  with  the  degree  of  the  injury.  Complete 
recovery,  as  a  rule,  rapidly  ensues.  Muscular  recovery  commences 
at  a  time  which  varies  in  the  same  way.  In  cases  in  which  the  muscles, 
though  paralyzed,  retain  their  irritability  to  the  interrupted  current, 
recovery  commences  in  three  or  four  weeks,  sometimes  earlier,  and 
soon  becomes  perfect.  This  degree  of  injury  is  seen  most  often  as  the 
result  of  compression  of  the  musculospiral  nerve,  producing  sleep, 
anesthetic  or  crutch  paralysis.  If  the;  reactions  typical  of  incomplete 
division  are  present  a  much  longer  time  is  necessary. 

After  neurolysis,  or  when  the  nerve  has  been  relieved  from  any 
form  of  pressure,  recovery  follows  exactly  the  same  lines. 


300  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Injuries  to  Terminal  Branches. — Facial  paralysis  may  result  from 
injury  to  the  nerve  (a)  above  the  geniculate  ganglion,  (h)  between  the 
ganglion  and  the  point  at  which  the  chorda  tympani  is  given  off,  and 
(c)  below  this  point.  When  injured  at  (6)  taste  is  affected  over  the 
anterior  two-thirds  of  the  corresponding  side  of  the  tongue. 

Apart  from  the  so-called  "rheumatic"  affections  of  the  nerve  (Bell's 
palsy),  interference  with  the  function  of  the  nerve  in  the  middle  ear 
as  a  result  of  disease  or  operation  is  the  usual  cause.  The  nerve  may 
suffer  in  fractures  of  the  skull  primarily,  or  more  often  from  involve- 
ment in  callus.  Outside  the  skull  it  may  be  injured  during  operations 
in  the  parotid  region  or  in  the  removal  of  tuberculous  glands,  or 
from  forceps  pressure  during  childbirth;  in  most  of  these  cases  the 
"division"  is  incomplete  and  spontaneous  recovery  ensues. 

In  cases  of  incomplete  division  the  usual  treatment  directed  toward 
maintaining  the  nutrition  of  the  paralyzed  muscles  must  be  adopted. 
When  due  to  middle-ear  disease  it  is  an  indication  for  the  complete 
mastoid  operation. 

When  the  reaction  of  degeneration  is  present,  showing  that  com- 
plete division,  either  anatomical  or  physiological,  has  occurred,  the 
treatment  to  be  adopted  depends  on  the  cause.  If  it  follows  a  radical 
mastoid  operation,  the  sooner  operation  is  carried  out  after  the  wound 
has  healed  the  better;  in  cases  of  Bell's  palsy  it  is  justifiable  to  wait 
for  six  months.  If  the  nerve  is  divided  during  the  course  of  a  mastoid 
operation,  the  ends  should  be  adjusted  in  the  aqueduct,  when  restora- 
tion of  function  may  be  expected  unless  neuritis  is  set  up  as  the  result 
of  sepsis.  If  discovered  after  the  operation  the  electrical  reactions 
should  be  tested  at  the  end  of  a  fortnight;  if  the  reaction  of  degenera- 
tion is  present,  the  wound  should  be  opened  up  and  an  attempt  made 
to  adjust  the  ends.  If  this  fails,  nerve  anastomosis  must  be  under- 
taken. It  must  be  remembered  that  the  injury  during  mastoid  opera- 
tion is  in  most  cases  incomplete,  and  that  spontaneous  recovery  follows 
the  usual  non-operative  treatment. 

Spinal  Accessory  Nerve. — ^The  external  or  spinal  portion  of  this 
nerve  is  not  infrequently  divided  during  the  course  of  operation  upon 
the  neck,  particularly  during  the  removal  of  tui)erculous  glands.  In 
many  of  these  cases  the  branches  of  the  cervical  nerves  to  the  trapezius 
are  affected  at  the  same  time  producing  its  complete  paralysis.  The 
extent  of  supply  of  the  spinal  accessory  and  the  cervical  nerves  to  the 
trapezius  varies;  as  a  rule  the  upper  portion  of  the  trapezius  is  par- 
alyzed by  division  of  the  spinal  accessory  alone. 

Cervical  Rib. — Symptoms  usually  appear  in  early  adult  life  and 
are  due  in  most  cases  to  pressure  upon  the  lowest  trunk  or  nerve 
entering  into  the  plexus.  In  many  cases  they  consist  of  wasting  of  the 
intrinsic  muscles  of  the  hand,  most  marked  and  starting  in  those  of 
the  thenar  eminence;  if  of  long  standing  the  reaction  of  degeneration 
may  supervene.  In  other  cases  the  principle  complaint  is  pain  along 
the  ulnar  border  of  the  forearm  and  hand,  or  a  general  weakness  or 


PERIPHERAL  PALSIES  301 

heaviness  of  the  whole  limb  noticed  at  the  end  of  the  day.  Sensory 
changes  are  unusual  and  when  present  rarely  exceed  epicritic  loss. 

This  condition  should  be  thought  of  in  all  cases  of  "brachial  neu- 
ralgia," or  of  wasting  of  the  thenar  muscles.  The  ribs  can  usually 
be  felt  but  occasionally  can  only  be  demonstrated  by  x-ray  exami- 
nation. 

Although  a  cervical  rib  be  present  it  does  not  necessarily  mean  that 
it  is  the  cause  of  the  symptoms.  Several  cases  have  been  observed 
in  which  a  cervical  rib  was  removed  from  a  patient  suffering  from 
syringomyelia  to  which  the  symptoms  were  due.  Thomas  Murphy 
has  recorded  a  case  in  which  the  symptoms  were  due  to  the  pressure 
of  the  first  rib.    A  similar  case  is  reported  by  Sherren. 

Treatment. — ^Treatment  consists  in  removal  of  the  rib  together  with 
the  periosteum  covering  it,  followed  by  careful  after-treatment. 

The  Long  Thoracic  Nerve  (Nerve  of  Bell). — ^This  nerve  is  most 
often  injured  in  males  between  the  ages  of  twenty-five  and  forty, 
usually  on  the  right  side.  Generally  caused  by  prolonged  pressure 
in  the  supraclavicular  region,  it  occasionally  follows  violent  muscular 
efforts  and  direct  violence  applied  to  the  shoulder. 

Paralysis  of  the  serratus  magnus  rarely  occurs  alone;  it  is  usually 
combined  with  paralysis  of  the  lower  trapezius. 

The  Circumflex  Nerve. — ^Injury  to  the  circumflex  nerve  is  by  no 
means  so  common  as  is  usually  supposed.  It  has  been  said  to  follow 
direct  blows  on  the  point  of  the  shoulder,  but  in  most  cases  the  injury 
is  to  the  anterior  primary  division  of  the  fifth  cervical  nerve,  and  careful 
examination  will  show  that  the  spinati  also  are  affected.  In  other  cases 
the  wasting  of  the  deltoid  in  common  with  the  muscles  around  the  joint 
on  which  the  circumflex  injury  was  diagnosed  has  been  found  to  be  the 
result  of  a  traumatic  arthritis. 

Careful  examination  is  necessary  before  coming  to  a  decision  with 
regard  to  treatment;  testing  must  be  carried  out  for  all  forms  of  sen- 
sibility. If  there  is  no  loss  of  sensibility,  and  there  is  paralysis  of 
the  deltoid  with  the  reaction  of  degeneration,  it  is  extremely  improbable 
that  the  circumflex  nerve  is  injured.  If  the  signs  are  those  of  complete 
section  of  the  nerve,  the  age  of  the  patient  and  his  occupation  must  be 
taken  into  consideration;  in  some  cases  operation  can  be  avoided  by 
training  the  neighboring  muscles  to  take  the  place  of  the  deltoid. 

Ulnar  Nerve. — This  nerve  may  be  injured  at  the  elbow,  or  at  the 
wrist  above  or  below  the  point  at  which  the  dorsal  branch  is  given  off. 
While  it  may  suffer  in  any  part  of  its  course  from  injury  and  the  result 
of  penetrating  wounds,  these  are  more  usually  at  the  wrist.  At  the 
elbow  the  injury  is  due  in  most  cases  to  fracture  or  deformity,  recent 
or  old,  usually  the  latter,  and  to  dislocation  of  the  nerve. 

If  complete  recovery  is  to  be  obtained  the  paralyzed  muscles  must 
be  kept  relaxed.  Sherren  has  devised  for  this  purpose  an  aluminum 
splint  to  be  worn  on  the  dorsum  of  the  hand,  keeping  the  fingers  slightly 
flexed  at  the  metacarpophalangeal  and  extended  at  the  interphalangeal 


302  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

joints.  If  this  is  worn  the  onset  of  "claw-hand"  may  be  prevented 
and  function  be  completely  restored. 

The  condition  of  dislocation  of  the  ulnar  occurs  most  often  in  males 
between  the  ages  of  twenty  and  thirty.  The  symptoms  usually 
come  on  immediately  after  the  injury;  occasionally  some  time  elapses, 
the  frequent  injuries  to  which  the  nerve  is  subject  in  passing  over  the 
internal  condyle  causing  fibrosis.  In  a  few  instances  the  condition 
originates  without  injury;  in  these  patients  the  fascia  which  keeps  the 
nerve  in  place  is  gradually  stretched. 

Musculospiral  Nerve. — The  special  interest  in  connection  with  this 
nerve  lies  in  the  fact  of  its  frequent  injury  in  association  with  fractures 
of  the  humerus,  particularly  of  its  lower  or  middle  third. 

In  addition,  "crutch,"  "sleep,"  "bridegroom,"  and  "Saturday 
night"  paralysis  are  not  uncommon  as  the  result  of  direct  pressure 
upon  the  nerve. 

In  every  case  the  paralyzed  muscles  must  be  kept  relaxed  by  suitable 
splints,  and  in  no  case  must  "wrist-drop"  be  allowed.  Operation  is 
rarely  indicated  except  in  the  cases  complicated  by  fracture,  as  the 
injury  is  incomplete  and  the  recovery  is  usually  rapid. 

The  prognosis  is  better  than  after  injury  of  any  other  nerve.  In 
cases  in  which  neurolysis  is  necessary,  restoration  of  function  often 
commences  within  a  few  weeks.  Even  after  secondary  suture,  motor 
power  usually  returns  within  a  year. 

External  Popliteal  Nerve. — This  nerve  suffers  most  often  as  the 
result  of  direct  violence  as  it  passes  around  the  neck  of  the  fibula. 
When  primarily  injured  in  association  with  fracture,  operation  should 
be  undertaken  at  once,  for  the  nerve  not  infrequently  passes  between 
two  fragments. 

However  caused,  the  division  is  usually  incomplete,  but  recovery  is 
always  slow  and,  unless  great  care  be  taken,  often  imperfect.  It  is 
essential  to  keep  the  foot  at  right  angles  and  to  prevent  foot-drop. 

External  Cutaneous  Nerve. — Injury  to  this  nerve  is  important  in 
that  it  is  occasionally  the  precursor  of  Bernhardt's  disease.  The 
nerve  is  usually  involved  at  its  exit  from  the  deep  fascia  and  in  many 
cases  a  spindle-shaped  swelling  may  be  felt  here.  The  injury  causing 
it  may  be  long  continued  as  by  a  badly  fitting  truss  or  in  other  cases  it 
follows  a  sudden  strain. 


CHAPTER  VII. 
LESIONS  OF  THE  SPINAL  CORD. 

The  anatomy,  histology,  and  physiology  of  the  spinal  cord  will  be 
discussed  only  in  so  far  as  problems  of  localization  and  pathology  are 
concerned. 

A  large  group  of  disorders,  chiefly  limited  to  the  cord,  come  under 
review.     One  may  present  them  as  a  series  of  syndromes  which  affect : 

1.  Chiefly  the  peripheral  motor  neuron  (anterior  horn  cells). 

2.  Combinations  of  anterior  horn  cells  and  pyramidal  tracts. 

3.  Chiefly  pyramidal  tracts. 

4.  Chiefly  posterior  columns. 

5.  Sympathetic  cell  groups. 

6.  Central  gray. 

7.  Combined  and  diffuse  lesions. 

These  subdivisions  are  largely  arbitrary;  at  times  they  correspond 
to  clinical  entities,  so-called,  again  they  are  fortuitous  combinations. 
Thus  a  poliomyelitis  may  clearly  delimit,  i.  e.,  in  its  end-results,  a 
group  of  motor  neurons,  anterior  horn  cells,  while,  on  the  other  hand, 
spinal  syphilis  may  show  any  of  the  localizations  just  tabulated.  For 
pedagogic  purposes  the  following  general  scheme  may  be  of  value : 


Areas  Involved. 


1.  Anterior  horn  group 

No.  7.   (Fig.  151). 

2.  Combined    (1)    and 

(2)  pyramidal  tract 
groups  Nos.  6  and  7. 


3.  Pyramidal  tract 
lesions  No.  6. 


4.  Posterior    column 
lesions  Nos.  1  and  2. 


.  Sympathetic 
groups  No.  9. 

6.  Central  group  No.  4. 


7.  Combined,  general 
and  indiscriminate 
groups. 


Chief  Eyndrome. 

Varying  with  metameres  involved, 
paresis  or  paralysis  of  muscles, 
atrophy,  atony,  loss  of  reflex  in 
muscle  group,  R.  D. 

From  (1)  muscular  atrophies,  R.  D., 
from  (2)  increased  reflexes,  increased 
tonus,  contractural  tendencies 

Paresis  or  paralysis,  more  usually  en- 
tire side,  with  spasticity,  increased 
tonus,  increased  reflexes,  no  atrophy, 
no  R.  D.,  associated  movements  of 
opposite  side. 

If  unilateral,  Burdach's  column  only, 
with  deep  anesthesia,  hyperesthesia, 
ataxia  of  limbs  lying  above  lesion. 
Diminished  or  lost  reflexes;  when 
including  Goll's  columns,  deep  anes- 
thesia, superficial  hyperesthesia, 
below  site  of  lesion. 

Trophic  disturbances;  vascular  dis- 
turbances, changes  in  secretions, 
hair,  skin,   etc. 

[Dissociation    of    thermal    and    pain 
1     senses  from  tactile  discriminations. 


Showing  various  combinations  of  pre- 
ceding. 


General  Diagnostic  Symbol. 

[.  Acute    anterior   poliomyelitis. 
!.  Chronic  anterior  poliomyelitis. 
).   Muscular    atrophies    combined 
with  some  dystrophies. 

: .  Amyotrophic  lateral  sclerosis, 
p.  Some  multiple  scleroses. 

I.  Lateral  sclerosis. 

'.  Compression,  tumor,  Pott's  dis- 
ease, bony  growths,  traumatic 
lesions. 

!.  Most    multiple    scleroses. 

I.  Toxic  (pellagra,  anemia,  arterio- 
sclerotic and  senile  syndromes. 

).  Tabetic  syndromes. 
..  Toxic      (lathyric,  alcoholic) . 
!.  Rare  compressions. 
!.  Friedreich's      syndromes. 
1.  Agenetic     (cerebellar     ataxias) 
syndromes. 


Trophoneuroses 

Angioneuroses 

Dermatoses 


See 

Vegetative 
Nerv.  System 
Disease. 

Hematomyelic  syndromes. 

Syringomyelic  syndromes. 

Tumor,    destruction     or     com- 

presison. 
Diffuse  myelitis. 
Multiple  sclerosis. 
Syphilitic  meningomyelitis. 


304 


LESIONS  OF  THE  SPINAL  CORD 


A  study  of  the  cross-section  of  the  cord  will  bring  these  localizing 
factors  more  closely  into  view.  The  localizing  features  are  brought 
out  in  Figs.  11-16  and  19-22  (see  pp.  42,  43,  46,  47,  50,  51). 


Radices  dorsalis  /+/If 
Radices  cervicales- 
Posterior  Root  Zone 


cerebello  spinalis 
I'oslerior   g. 

Limiting  Layer- 


Iractus  cortico 
spinalis  cruciatus 


Tractus  cerebello' 
spinalis  anterior 


Radices  dorsales  V+XII 
Radices  lumbares 
I  Rfidices  sacrales^-^<>^"  I  -f^eW 

,  3\     __^<;::     2     /Descending  dorsal  root  fibers 
-^yiarginal  Zone 

'ractus  corticospinalis 


ractus  thalamo- 
spinalis 


Fibrac  associativae  breves 

librae  associativae  longae 


Fig.   151. — -Cross-section  of 


Ventral  Field 
Fasc-  olivares 


ractus  tectospinalis 
cruciatus 

Fasciculus  marginalis 
'ractus  corticospinalis 
Fasciculus  sulcomarginalis 
Fasciculus  longitudinalis  dorsalis 

spinal  cord  showing  localization  of  chief  structures  with 
lesions.     (Veraguth.) 


Location  of  Lesion. 
1.  In  the  posterior  root  zone. 


2.  In  posterior  column  of  one  side. 


3.  In  Goll's  columns  of  both  sides. 


4.  In  central  gray,  especially  of  anterior 

commissure. 

5.  Posterior  portion  of  the  lateral  columns 

with  integrity  of  limiting  layer. 

6.  Pyramidal  tracts. 


7.  Anterior  horns. 


8.  Spinocerebellar  paths. 

9.  Lateral  recess. 


Chief  Symptoms. 

Irritation  causes  hyperesthesise.  Destruc- 
tion causes  loss  of  superficial  sensibility 
in  the  root  distribution  spreading  over 
at  least  three  roots.  Ataxia  and  event- 
ually astereognosis  in  the  extremity 
involved. 

Anesthesia  to  deep  sensibility  and  to 
touch.  Ataxia  of  metameres  below  the 
lesion. 

Anesthesia  to  deep  sensibility  and  hypes- 
thesia  of  the  lower  extremities  only, 
even  in  high  lesions. 

Dissociated  sensibility  (thermanesthesia 
and  analgesia  in  the  affected  metamere 
as  indicated  in  the  skin  distribution). 

Crossed  hemihypesthesia  plus  the  symp- 
toms of  6. 

Spastic  paralysis  of  the  caudal  metameres 
below  the  lesion  without  reaction  of 
degeneration,  often  crossed  movements, 
no  atrophy  and  with  increased  reflexes. 

Flaccid  paralysis  of  the  muscles  of  a  num- 
ber of  root  zones,  atropia  and  atrophy 
of  muscles  of  involved  metameres; 
R.  D.,  loss  of  reflexes. 

Bilateral  involvement  causes  cerebellar 
ataxia. 

Sympathetic  disturbances  metamericaUy 
distributed. 


The  majority  of  these  cord  syndromes  are  considered  here,  some  are 
discussed  under  their  etiological  groupings:  syphilis  of  the  nervous 
system,  for  instance,  taking  over  tabes,  spinal  cord  gummata,  syphilitic 
meningomyelitis. 


ACUTE  POLIOENCEPHALOMYELITIS  305 

ACUTE  POLIOENCEPHALOMYELITIS. 

Historical  Note. — ^This  disease  has  affected  mankind  for  centuries. 
Mitchell  reports  shortening  of  the  femur,  presumably  due  to  this 
disease,  in  an  Egyptian  mummy.  Jonathan's  son  (II  Samuel,  iv,  4.) 
possibly  had  this  disease.  Numerous  drawings  and  paintings  of  the 
fourteenth  and  fifteenth  centuries  (Hieronymus  Bosch)  attest  to 
its  prevalence  in  these  times.  Throughout  the  period  of  the  later 
Latin  writers  it  was  usually  included  under  paralyses,  hemiplegias, 
etc.  It  was  Underwood  who,  in  1784,  described  a  new  disease 
"Debility  of  the  Lower  Extremity,"  that  gave  the  first  impetus  which 
led  to  its  definite  separation  as  a  type  by  Jacob  v.  Heine  in  1840. 
In  1810  Jorg  gave  an  excellent  case  history,  and  in  184.3,  independent 
of  Heine,  Rilliet  and  Barthiez  contributed  an  important  article  to 
French  literature.  They  called  the  disease  an  Essential  Paralysis, 
and  thought  it  had  little  spinal  pathology. 

Although  much  was  written  previous  to  Duchenne's  time,  his  work 
in  1855  marked  the  beginning  of  a  new  era  in  the  study  of  this  disease 
and  in  Heine's  second  edition,  1860,  the  status  of  the  disease  at  that 
time  is  well  reflected.  In  1865  attention  was  first  called  to  the  possible 
relation  of  infantile  to  adult  poliomyelitis  by  M.  Meyer,  which  study 
was  followed  by  a  large  number  of  further  contributions  from  Charcot's 
students. 

The  anatomical  era  may  be  said  to  have  opened  with  Cornil  in  1863, 
and  there  then  grew  up  the  Charcot  thesis  of  a  primary  affection  of  the 
ganglion  cells  of  the  anterior  horns,  which  has  had  to  give  way  to  the 
more  recent  studies  of  Wickmann,  Harbitz  and  Scheel,  Flexner,  Strauss, 
and  others  (1807-1910). 

Seeligmiiller's  masterly  monograph  in  1880  practically  contained 
the  standard  teachings  up  to  the  appearance  of  Medin's  work  in 
1896,  when  the  epidemiological  features  of  the  disease  were  brought 
out.  In  later  years  Lovegren  (1904),  Wickmann  (1905-1907),  Harbitz 
and  Scheel  (1905)  have  still  further  refined  the  clinical  and  pathological 
aspects,  while  Flexner,  Landsteiner,  Popper,  and  others  have  been 
able  to  convey  the  disease  from  the  human  to  monkeys,  and  thence 
to  other  monkeys  (1909-1911). 

The  most  recent  monographic  treatment  of  the  subject  is  found  in 
Wickmann 's  very  able  contribution  in  the  Handbuch  der  Neurologie, 
1911,  and  Miiller's  equally  valuable  monograph  (1911),  one  by  Romer^ 
(1911),  and  by  Peabody  and  Draper  (1913). 

Etiology. — The  disease  is  an  acute  infectious  disease.  The  organism 
is  probably  a  living  one;  it  can  be  conveyed  to  human  beings,  to 
monkeys  and  to  rabbits.  A  small  amount  of  the  emulsion  of  the  spinal 
cord  of  humans  injected  into  the  brain  of  a  monkey  has  caused  the 
disease,  which  Flexner  has  transmitted  from  monkey  to  monkey  for 

1  Translated  in  Nervous  and  Mental  Disease  Monograph  Series,  New  York. 
20 


306  LESIONS  OF   THE  SPINAL  CORD 

twenty-five  generations.  The  organism  is  thought  to  be  a  small 
organism,  capable  of  passing  through  a  Berkefeld  filter,  and  one  whose 
virus  is  destroyed  by  heat  and  weak  disinfectants,  but  not  by  cold  nor 
dryness.  In  many  respects  the  virus  resembles  that  of  rabies.  It 
has  been  conveyed  by  direct  injection  into  the  brain,  through  the 
uninjured  and  scarified  nasal  mucous  membrane,  and  through  injection 
of  the  intestinal  lymph  glands  of  afi^ected  animals  (Flexner). 

An  immunity  of  yet  unknown  length  seems  to  be  established  by 
one  attack.  The  disease  seems  to  be  conveyed  by  direct  contact, 
through  indirect  contact,  and  through  nasal  and  gastro-intestinal 
secretions.     It  does  not  seem  to  be  highly  contagious. 

Epidemic  extensions  have  now  been  studied  for  nearly  sixty  years, 
and  Cordier  first  expressed  a  belief  in  its  contagiousness.  Medin  first 
definitely  proved  it.  Some  eighty  or  more  epidemics  have  been 
reported  to  1912.  The  most  recent  pan-epidemic  apparently  started 
in  Norway  and  Sweden  in  1903-1904,  spread  to  the  United  States  in 
1907-1912,  to  Germany  and  France  in  1908-1911,  with  isolated  far- 
lying  outbreaks  in  Cuba,  Australia,  etc. 

Rural  districts  have  suffered  greatly,  and  density  of  population  has 
not  seemed  to  play  a  large  role;  cold  climates  seem  more  favorable, 
and  the  season  is  usually  in  the  warm,  dry  months. 

The  majority  of  the  cases  occur  in  children  from  one  to  five  years  of 
age,  with  lower  limits  at  tw^o  weeks,  and  over  sixty  years  as  outlying 
rarities.  Certain  epidemics  have  shown  marked  variability  in  the 
matter  of  age  incidence,  the  Swedish  epidemic  of  1905  having  as  high 
as  10  per  cent,  adults.  In  large  epidemics  adults  seem  more  often 
affected.  The  male  sex  has  seemed  to  be  more  often  involved,  but  the 
differences  are  not  very  marked.  Nationality  seems  to  play  little  role, 
although  it  has  been  assumed  that  Scandinavians  are  peculiarly 
susceptible.  This  may  only  be  an  indication  of  the  more  careful  study 
given  by  these  authors.     Neuropathic  heredity  seems  to  play  no  role. 

The  incubation  period  varies  from  one  to  ten  days,  the  majority 
showing  a  period  of  from  one  to  five  days.  In  experimental  polio- 
myelitis of  monkeys  the  incubation  time  is  about  six  to  nine  days. 

Pathology. — The  older  concepts  of  a  primary  intoxication  or  of  an 
inflammation  of  the  motor  horn  cells  exclusively  must  be  abandoned. 
Acute  poliomyelitis  is  due  to  an  acute  interstitial  inflammation  (a 
meningomyelitis),  involving  all  parts  of  the  cord.  There  is  a  conges- 
tion, infiltration,  and  edema  of  the  leptomeninges,  cord,  medulla,  pons, 
cerebellum,  and  cerebrum.  The  dura  is  not  markedly  involved; 
the  pia  is  congested  and  infiltrated  with  round  mononuclear  cells 
(lymphocytes),  particularly  in  the  sacral  and  lumbar  region.  The 
vessels  are  congested  and  their  sheaths  infiltrated,  the  progression 
of  the  inflammatory  reaction  apparently  following  the  vessels  from 
the  periphery  within  the  cord.  The  cerebrospinal  fluid  is  increased  in 
quantity,  almost  opalescent  early  in  the  disease,  with  markedly 
increased  lymphocytes,  in  some  of  which  Lafora  and  Hough  have 


ACUTE  POLIOENCEPHALOMYELITIS 


307 


found  pictures  resembling  the  Leishman-Donovan  bodies.  The  fluid 
later  becomes  clearer,  but  still  shows  a  pathological  increase  in 
leukocytes. 

Within  the  cord  the  inflammatory  process  follows  the  pial  processes 
into  the  depths  of  the  anterior  fissure  and  along  the  sheaths  of  the 
central  vessels.  The  posterior  root  fibers  and  the  spinal  ganglia  are 
also  infiltrated. 

The  vascular  lesions  are  particularly  noticeable,  and  the  interstitial 
and  ganglionic  changes  depend  largely  upon  them.  The  vessels 
throughout  are  dilated  and  engorged,  the  capillaries  often  being 
enormously  distended.     This  marked  hyperemia  is  found  throughout, 


Fig.  152. — Acute  poliomyelitis,  showing  A, 
associated  meningitis;  B,  edge  of  cord. 


Fig.  153. — Acute  poliomyelitis, 
showing  the  vascular  congestion  and 
the  surrounding  infiltration.  The 
anterior  spinal  artery. 


and  vascular  hemorrhagic  lytic  changes  are  frequent  but  small.  As 
in  rabies,  and  to  some  extent  in  syphilis,  there  is  a  marked  perivascular 
or  intra-adventitial  infiltration,  apparently  of  lymphocytes,  not  plasma 
cells.  Interstitial  changes  in  the  gray  and  white  matter,  chiefly  of  an 
infiltration  of  cells,  and  proliferation  of  glia  cells,  occur.  By  reason  of 
the  rich  blood  supply  the  gray  matter  of  the  anterior  horns  bears  the 
brunt  of  the  inflammatory  edema  and  hyperemia,  with  destruction  of 
many  of  its  motor  cells.  This  is  a  secondary  process.  Harbitz  and 
Scheel  believe  that  small  abscesses  take  place,  but  this  is  an  exception 
if  it  does  occur. 

The  ganglion  cells  undergo  varying  degrees  of  degeneration,  some- 
what proportioned  to  the  infiltration,  the  axis-cylinder  finally  breaking 


308 


LESIONS  OF  THE  SPINAL  CORD 


down.  Amid  the  degenerated  cells  many  normal  ones  may  be  found, 
and  the  distribution  of  degenerated  areas  is  very  variable,  the  sacro- 
lumbar  cord  being  more  seriously  affected,  although  any  part  of  the 
cerebrospinal  axis  may  be  involved.  All  classes  of  ganglion  cells  go 
under,  but  the  lateral  dorsal  cord  regions  are  much  less  involved; 
however,  many  vegetative  system  cells  are  injured. 

As  a  rule  the  functional  involvement  is,  by  reason  of  the  edema, 
hyperemia,  and  infiltration,  far  in  excess  of  the  permanent  anatomical 
loss,  hence  the  widespread  character  of  the  paralysis  in  the  early 
stages  and  the  marked  degree  of  recovery  possible.  Only  a  small 
proportion  of  the  primarily  involved  ganglion  cells  degenerate  entirely. 


Fig.   154. — Acute  poliomyelitis.     The  various  stages  of  destruction  of  the 
anterior  horn  cells. 


The  fiber  pathways  may  show  permanent  infiltration  changes  with  the 
production  of  anomalous  spastic  phenomena. 

The  spinal  nerves  are  involved  usually  at  their  junction  with  the 
cord,  and  some  polyneuritis  may  be  present  early  in  the  disease. 

The  medulla,  pons,  cerebellum,  and  cerebrum  all  are  involved 
to  a  greater  or  lesser  degree.  Certain  cases  show  that  the  main 
lesion  is  in  one  or  more  of  these  regions,  rather  than  in  the  spinal  cord. 
True  encephalitis,  with  varying  degrees  of  weak-mindedness,  is  the 
frequent  result  of  these  higher  lying  types. 

The  other  organs  of  the  body  do  not  escape.  There  are  evidences 
of  an  acute  general  infection  everywhere.^ 


1  Walter,  Deut.  Zeits.,  vol.  xlv,  No.  2. 


ACUTE  POLIOENCEPHALOMYELITIS  309 

Symptoms. — The  study  of  the  recent  epidemics  has  shown  a  great 
variabiHty  in  the  affection,  but  practically  all  of  the  cases  show  the 
effects  of  an  acute  infection  as  prodromal  and  early  signs.  The  later 
course  of  the  disease,  dependent  upon  the  locations  chiefly  involved, 
permits  a  separation  into  several  types  of  which  Wickmann  recognizes 
eight  classical  pictures. 

Prodromata  are  usual.  They  vary  in  the  different  epidemics. 
They  consist  of  fatigability,  loss  of  appetite,  slight  digestive  disturb- 
ances, with  nausea,  looseness  of  the  bowels,  coryza  or  bronchial  irrita- 
tion (bronchitis,  bronchopneumonia)  with  slight  elevation  in  tem- 
perature. Conjunctivitis  may  occur;  lymphatic  swellings  are  usual. 
The  patient  may  thus  suffer  for  twenty-four  to  seventy-two  hours 
before  the  acute  prostrating  effects  of  the  disease  become  manifest. 
Some  few  cases  show  almost  complete  recovery  after  such  prodromata, 
and  then  are  again  taken  ill  suddenly.  Careful  observation  will 
probably  reduce  the  number  of  cases  reported  as  coming  on  without 
prodromata. 

The  cerebrospinal  fluid  in  the  prodromal  stages  may  show  opal- 
escence with  very  marked  lymphocytosis.  The  blood  changes  are 
apparently  not  constant.  La  Fetra  has  reported  a  leukocytosis 
of  from  13,000  to  20,000;  whereas  Miiller  has  found  a  leukopenia  of 
from  3000  to  5000,  and  also  finds  leukopenia  in  experimental  monkey 
poliomyelitis^  The  lymphocytes  are  increased.  No  parasites  have 
been  found  in  the  blood  up  to  the  present  time  (Dickson). 

After  twenty-four  to  seventy-two  or  more  hours  the  temperature 
suddenly  rises.  It  varies  from  99°  F.  to  102.5°  F.  or  even  105°  F.  to 
106°  F.,  and  bears  little  relation  to  the  severity  of  the  disease.  Abor- 
tive cases  have  shown  high  temperatures,  and  severe  cases  little.  Chills 
and  convulsions  occasionally  attend  the  rise  in  temperature.  The 
temperature  curve  is  not  characteristic,  being  either  remittent  or 
continuous.  Subnormal  temperatures  occur,  and  indicate  marked 
cervical  involvement. 

Headache  is  a  frequent  symptom.  It  is  often  severe  and  usually 
frontal;  it  may  be  occipital,  resembling  a  meningeal  headache.  Pros- 
tration is  marked,  diarrhea  is  extremely  common,  vomiting  is  frequent, 
and  constipation  is  not  unusual.  Respiratory  symptoms  are  not 
marked,  save  when  respiratory  palsies  occur.  Here  dyspneas  are  the 
rule.  The  kidneys  show  little,  and  the  heart's  action  is  that  of  an 
acute  infection  in  general,  with  a  tendency  to  show  nervous  tachy- 
cardia, especially  in  the  cases  with  medullary  involvement.  Weakness 
is  thcrule. 

Early  profuse  sweating  is  a  frequent  symptom  and  skin  eruptions 
are  occasionally  seen,  although  herpes  is  rare  in  contrast  with  cerebro- 
spinal meningitis.     Other  exanthemata  occasionally  appear. 

Nervous  Symptoms. — As  a  rule  there  is  considerable  restlessness, 
especially  in  older  patients.  The  children  are  peevish,  petulant,  or 
very  fretty.     Sleep   is   often   disturbed,   with  frequent   crying   out. 


310  LESIONS  OF  THE  SPINAL  CORD 

Some  children  lie  drowsy  or  apathetic,  and  may,  though  rarely,  show 
coma,  delirium,  or  convulsions.  Pain  is  a  frequent  early  symptom, 
and  may  be  very  severe.  Marked  hyperesthesia  is  usual  (90  per  cent.), 
and  is  brought  on  by  the  least  attempt  at  motion  of  the  limbs,  pressure 
on  the  nerve  trunks,  touching  of  the  skin.  Heine  called  attention 
to  this  in  1840.  Movements  of  the  head  and  spine  are  particularly 
painful,  and  some  patients  are  extremely  anxious  and  fearful,  whimper- 
ing continuously,  closely  resembling,  in  the  early  stages,  meningeal 
cases.     There  is  frequent  photophobia  and  hyperacusis. 

The  infiltration  in  the  cord  causes  other  sensory  symptoms,  such  as 
paresthesise  and  anesthesise.  Stiffness  is  not  infrequent,  with  slight 
though  not  marked  retraction  of  the  head  (Kernig's  sign)  in  some  and 
varying  degrees  of  rigidity  of  the  limbs,  with  contracted  position  of 
the  lower  limbs  particularly,  are  frequent. 

Twitching  and  jerking  of  the  limbs  are  also  frequent. 

The  stage  of  paralysis  soon  sets  in  and,  according  to  the  predominant 
localization,  following  Wickmann,  eight  types  may  be  distinguished. 
These  types  represent  general  tendencies  rather  than  hard-and-fast 
differences.  The  further  symptomatology  will  be  given  in  accord- 
ance with  these  divisions.  These  types  are:  (1)  spinal  poliomyelitis 
forms  (the  commonest  type);  (2)  acute  ascending  types  (Landry's 
paralysis);  (3)  bulbar  or  pontine  forms;  (4)  encephalitic  forms;  (5) 
ataxic  forms;  (6)meningiticforms;  (7)neuriticforms;  (8)  abortive  forms. 

1.  Spinal  Forms. — ^There  is  usually  an  early  prodromal  weakness, 
or  even  a  paresis  which  is  very  widespread.  This  develops  to  a  rela- 
tively marked  paresis,  sometimes  within  twelve  to  twenty-four  hours, 
but  more  often  in  from  two  to  five  days,  occasionally  after  a  week. 
Careful  observation  has  shown  that  the  paralysis  is  a  gradual,  rather 
than  an  abrupt  one.  It  begins  with  weakness,  advances  to  paresis, 
and  finally  becomes  a  definite  paralysis,  whereas  the  weakness,  how- 
ever, is  very  widespread,  the  palsies  are  less  so,  and  the  paralyses 
even  more  restricted.  When  the  ultimate  stages  are  reached,  the 
weaknesses  clear  up,  the  palsies  gradually  disappear,  and  the  residual 
paralyses  often  represent  but  a  small  part  of  what  appeared  to  be  a 
wholesale  devastation.  This  is  in  strict  accordance  with  the  pathological 
features. 

The  distribution  of  paralyses  is  due  to  factors  concerning  which 
there  is  little  definite  information.  From  a  purely  statistical  study 
of  the  cases  it  has  been  found  that  the  lower  limbs  are  involved  twice 
as  often  as  the  upper;  in  some  epidemics  four  times  as  often.  The 
entire  limb  is  rarely  involved  permanently,  but  special  muscle  groups 
are  picked  out.  Thus  in  the  lower  extremities  the  quadriceps,  the 
peronei,  and  the  tibialis  anticus  are  the  oftenest  affected;  in  the  upper 
extremities  the  scapular  and  deltoid  muscles.  Asymmetry  in  the 
final  picture  is  the  rule.  The  muscles  of  the  trunk  are  involved  next 
most  frequently,  while  the  arms  are  least  frequently  involved.  The 
trunk  muscle  palsies  are  often  overlooked,  however. 


ACUTE  POLIOENCEPHALOMYELITIS  311 

In  young  children  it  is  almost  impossible  to  localize  the  paralyses 
in  the  early  stages,  especially  as  many  such  patients  go  through  the 
initial  stages  of  the  disease  without  there  being  a  suspicion  of  the  real 
difficulty.  Here  the  loss  of  the  reflexes,  the  hypotonus,  the  careful 
scrutiny  of  the  position  of  the  limbs,  the  behavior  to  passive  motion 
and  resistance  movements  and  the  tickling  responses  lead  to  a  correct 
appreciation  of  the  difficulties.  They  also  permit  a  diagnosis  of  rudi- 
mentary and  of  mild  cases.  Babies  in  arms  behave  as  though  they 
were  lumps  of  dough,  and  the  mother  notices  the  hypotonus. 


Fig.  155. — Abdominal  muscle  palsy — poliomyelitis.     (Frauenthal.) 

Weakness  of  the  muscles  of  the  abdomen  is  an  important  early 
diagnostic  feature,  especially  in  the  differentiation  from  meningitis. 
The  involvement  is  usually  bilateral  and  diffuse.  The  muscles  are 
hypotonic,  and  swell  out  as  though  the  intestines  were  inflated  with 
gas.  There  is  often  a  loss  of  the  epigastric  and  abdominal  reflexes — 
symmetrical  or  assy  metrical.  The  patients  are  further  unable  to  come 
from  a  horizontal  to  a  sitting  position.  In  some  patients  the  abdominal 
muscles  are  alone  involved.  Obstinate  constipation  usually  accom- 
.  panics  the  abdominal  palsies. 

Of  the  back  muscles  the  latissimus  dor  si  are  the  oftenest  involved. 
The  glutei  are  also  somewhat  implicated.    Children  with  these  palsies 


312  LESIONS  OF  THE  SPINAL  CORD 

waddle  when  they  walk.  They  behave  like  children  with  muscular 
dystrophy  on  rising  from  the  floor. 

Only  rarely  is  the  diaphragm  implicated.  They  are  usually  among 
the  fatal  cases.  Unilateral  diaphragmatic  palsies  have  been  observed. 
The  muscles  of  the  hips  are  involved  in  at  least  tw^o-thirds  of  the  cases. 

The  following  table  from  Wickmann  will  serve  to  indicate  the 
general  run  of  the  cases.  The  figures  represent  the  study  of  868 
cases  in  the  Swedish  epidemic  of  1905: 

1.  Paralysis  of  one  or  both  legs 353 

2.  Paralysis  of  one  or  both  arms 75 

3.  Combined  paralyses  of  arms  and  legs 152 

4.  Combined  leg  and  thigh  paralyses 85  ' 

5.  Combined  arm  and  thigh  paralyses ■ .      .      .      .  10 

6.  Isolated  thigh  paralysis 9 

7.  Paralysis  of  the  entire  body 23 

8.  Ascending  paralysis 32 

9.  Descending  paralysis 13 

10.  Combination  of  spinal  and  cranial  nerve  paralyses      ....       34 

11.  Isolated  cranial  nerve  palsies 22 

12.  Localization  uncertain 60 

868 

The  figures  of  the  Committee  of  the  New  York  Neurological  and 
New  York  Pediatric  Societies  give  similar  results.^ 

The  bladder  is  frequently  involved  in  the  early  stages.  Urinary 
retention  is  frequent,  incontinence  is  rare.  As  a  rule,  the  disturbance 
is  transitory,  hence  overlooked.  It  is  frequently  thought  to  be  a  symp- 
tom of  the  general  infection,  but  Wickmann  regards  it  as  due  to  central 
nervous  lesions. 

Constipation  is  not  unusual,  but  marked  disturbances  of  the  intestinal 
functions  are  rare. 

Sensory  Disturbances. — ^The  older  teachings  that  sensory  disturb- 
ances are  uniformly  absent  is  not  true.  Almost  invariably  there  is 
in  the  beginning  of  the  disease  a  marked  hyperesthesia.  The  slightest 
touch  causes  marked  reaction.  Loss  or  diminution  of  sensation  is  also 
not  uncommon.  Complete  anesthesia  is  rare,  but  has  been  observed. 
Hypesthesise  are  common,  and  may  include  both  thermal  and  pain 
sensibility.  In  young  children  the  difficulties  in  observation  cause 
one  to  overlook  these  sensor}'  anomalies. 

Atrophic  Stages.— For  a  variable  length  of  time,  a  year  at  least,  the 
palsied  or  paralyzed  muscles  gradually  regain  their  form  and  their 
function,  but  sooner  or  later,  depending  on  the  grade  of  central 
involvement,  a  more  or  less  permanent  state  of  inactivity  is  reached — 
a  residual  period  in  which  the  final  account  of  stock  may  be  taken. 
In  this  stage  one  meets  with  the  definite  atrophies,  the  beginning  of 
the  various  deformities,  changes  in  the  bones  and  joints,  and  the 
residual  secretory  and  trophic  anomalies. 

1  Nervous  and  Mental  Disease  Monograph  Series  No.  7,  New  York,  1910. 


ACUTE  POLIOENCEPHALOMYELITIS  313 

The  various  deformities  belong  more  in  the  domain  of  orthopedic 
sm-gery  and  cannot  be  taken  up  here.  Pes  equinus,  pes  calcaneus, 
pes  valgus,  pes  varus,  hyperextension  of  the  knee-joint,  genu  valgum, 
genu  varum,  scolioses,  kj-phoses,  lordoses,  torticollis,  flail  arm,  etc., 
are  among  the  more  common  residual  deformities.  Seeligmiiller's 
monograph,  already  cited,  treats  of  them  at  great  length. 

The  permanent  vasomotor  disturbances  are  chiefly  cold  extremities 
and  cyanosis.    Dryness  of  the  skin  is  frequent. 

2.  Acide  Ascending  Form — Landry's  Paralysis. — Landry,  in  1859, 
described  an  acute  ascending  paralysis,  which  later  study  has  sho^vTi  to 
be,  for  the  most  part  at  least,  a  true  polioencephalomyelitis.  Here 
the  palsy  shows  itself  usually  flrst  ui  the  lower  extremities,  then  the 
muscles  of  the  hip,  the  abdomen,  the  thorax,  and  the  cranial  nerves, 
are  involved  and  death  generally  occurs  through  implication  of  the 
cardiac  and  respiratory  centers.  Death  takes  place  in  from  foiu- 
to  five  days,  usually  with  clear  consciousness,  or  snght  coma.  The 
sensibility  is  usually  intact,  or  only  slightly  dulled. 

Occasionally  the  patients  recover,  and  then  show  the  residuals  of 
a  severe  spinal  poliomyelitic  type,  with  mixture  of  bulbar  or-  ponthie 
features. 

A  descending  form  is  also  to  be  distinguished.  This  is  much  rarer. 
Here  the  bulbar  symptoms  develop  early,  and  the  spmal  extension 
shows  later. 

The  majority  of  the  fatal  cases  of  epidemic  polioencephalomyelitis 
show  the  type  of  a  Landry  paralysis. 

3.  Bidhar  and  Pontine  Forms. — Here  the  features  that  stand  in 
the  foregroimd,  either  as  initial  or  as  residual  conditions,  are  the 
cranial  nerve  palsies.  A  large  number  of  the  patients  v^-ith  poliomyelitis 
show  some  cranial  nerve  complications,  but  when  these  are  the  chief 
features,  and  the  spinal  palsies  are  the  minor  complicating  factors, 
then  one  speaks  of  the  bulbar  and  pontine  t>-pes. 

Many  of  the?e  patients  show  isolated  palsies — others  have  two  or 
more  cranial  nerve  involvements.  The  facial  is  oftenest  implicated 
(10  per  cent.),  next  the  hypoglossal.  These  are  usually  unilateral, 
although  Medin  has  described  a  bilateral  facial  palsy.  Eye  muscle 
palsies  are  infrequent.  The  third  and  fourth  less  often  than  the  sLxth 
(Wickmann,  Miiller). 

Ptosis,  ophthalmoplegia  interna,  externa,  nystagmus  are  among 
the  rarities.  Pupillary  distm-bances  are  not  infrequent,  according 
to  Lundborg.  Here  both  light  and  accommodation  reflexes  are  inter- 
fered with.  Very  rarely  one  observes  amaurosis  ^s'ith  optic  nerve 
involvement.  The  presence  of  choked  disk  in  meningitis  is  an  impor- 
tant differential.  The  IXth,  Xth,  Xlth  are  involved,  usually  in  the 
fatal  cases,  occasionally  unilateral  phar\TLgeal  and  larjTigeal  palsies  are 
residual  conditions.  Speech  distm-bances  are  present,  but  infrequent. 
Midbrain  involvements  with  peculiar  tremors,  vertigoes,  rotatory 
movements,  ataxias,  etc.,  are  among  the  curiosities. 


314 


LESIONS  OF  THE  SPINAL  CORD 


4.  Encephalitic  or  Cerebral  Form,. — Striimpell  called  particular 
attention  to  the  possibility  of  a  pure  encephalic  form  of  the  disease, 
thus  widening  the  conception  from  a  poliomyelitis  to  a  polioencephalo- 
m^^ehtis.  Medin's  valuable  study  confirmed  his  teaching,  and  the 
investigations  of  recent  years  have  still  further  amplified  the  find- 
ings. Here  the  inflammatory  reaction  spreads  throughout  the  entire 
cortex,  as  in  the  cord.  The  central  and  frontal  gyri,  the  basal  ganglia, 
the  internal  capsule,  and  centrum  ovale  are  most  frequently  involved. 

Here,  in  addition  to  headache,  stupor,  and  convulsions  one  encounters 
spastic  palsies,  hemiplegic  or  diplegic  in  type,  usually  associated  with 
bulbar  palsies. 


Fig. 


156. — Eye  palsies,  poliomyelitis. 
(Frauenthal.) 


Fig.  157. — Facial  nerve  palsy,  polio- 
myelitis.    (Frauenthal.) 


These  cerebral  forms  are  probably  rare,  and  often  fatal.  They 
are  also  rare  in  experimental  monkey  poliomj^elitis.  Miiller  is  inclined 
to  regard  the  spastic  palsies  that  occasionally  occur  as  due  to  pontine 
rather  than  to  motor  cortex  involvements,  whereas  Wickmann  lays 
particular  stress  upon  the  probabilities  of  their  cortical  origin. 

5.  Ataxic  Forms. — Medin  described  forms  in  which  the  patients 
showed  ataxia  in  walking,  with  staggering  or  ataxic  gaits,  and  others 
behaving  like  Friedreich's  disease  of  acute  onset.  These  forms  are 
closely  allied  to  the  bulbar  and  pontine  types,  and  also  may  be  oc- 
casioned by  extension  of  the  disease  to  the  cerebellum.  The  latter 
structure  is  almost  always  involved  .to  some  extent  in  this  disease. 

6.  Meningitic  Forms. — Here  meningeal  symptoms  occupy  the  fore- 
ground. Headache,  vomiting,  pain  in  the  neck  with  stiffness,  Kernig's 
sign,  stiffness  of  the  back,  opisthotonos,  convulsions,  strabismus, 
somnolence,  and  unconsciousness  are  present.    These  cases  either  then 


ACUTE  POLIOENCEPHALOMYELITIS  315 

develop  marked  spinal  and  bulbar  symptoms  of  the  ordinary  type,  or 
the  symptoms  recede  with  either  minor  residuals,  eye  palsies,  etc.,  or 
go  on  to  complete  recovery. 

7.  Polyneuritic  Forms. — The  study  of  recent  epidemics  has  shown 
the  great  frequency  with  which  pain  is  found  in  the  initial  history. 
In  many  cases  there  are  painful  nerve  trunks,  with  Lasegue's  phenom- 
enon, and  great  hyperalgesise  over  the  entire  body,  resembling  polyneu- 
ritis. Anatomically,  however,  marked  neuritic  changes  are  wanting. 
These  cases  Wickmann  prefers  to  call  neuritis-like. 

8.  Abortive  Forms. — ^The  study  of  the  epidemics  of  1909-1912  has 
shown  that  in  a  number  of  patients  the  illness  began  with  the  charac- 
teristic symptoms  of  poliomyelitis,  and  then  went  on  to  recovery  with- 
out any  palsies.  In  others  again,  widespread,  though  mild,  palsies 
with  hypotonia  developed  and  total  recoveries  occurred  within  a  short 
time.  Wickmann  brought  these  facts  into  prominence,  and  showed 
that  these  were  to  be  regarded  as  abortive  cases.  The  most  frequent 
forms  under  which  these  cases  develop  are  (1)  that  of  a  mild  meningitic 
type  with  the  usual  prodromal  signs,  associated  with  the  neck  symp- 
toms, stiffness,  pains,  sometimes  opisthotonos  and  the  like;  (2) 
cases  with  the  symptoms  of  a  general  infection  only;  (3)  cases  running 
a  course  like  an  influenza;  (4)  cases  with  marked  gastro- intestinal 
signs. 

Wickmann  has  estimated  that  at  least  15  per  cent,  of  all  the  cases 
can  be  grouped  under  this  rubric,  while  Miiller  believes  them  to  occur 
much  oftener,  indeed,  more  often  than  the  usual  type.  Their  signi- 
ficance in  the  epidemiology  of  the  disease  is  great,  because  it  is  highly 
probable  that  its  spread  may  be  conditioned  by  these  ambulatory 
abortive  cases.  They  are  more  frequent  in  children,  but  may  also 
be  present  in  adults.  If  Miiller's  stand  be  correct,  viz.,  that  they  occur 
oftener  than  the  well-developed  forms,  their  importance  from  this 
stand-point  is  enormous.  The  question  of  rarity  of  the  disease  after 
puberty  may  be  solely  conditioned  by  the  fact  of  previous  abortive 
attacks  in  many  individuals.  Miiller  states  that  in  the  study  of  small 
epidemics  he  has  found  that  in  the  abortive  cases  there  is  a  marked 
tendency  to  show  the  same  symptoms  in  their  epidemic  extension. 
Thus  gastro-intestinal  cases  give  rise  to  gastro-intestinal  cases, 
respiratory  to  respiratory,  meningeal  to  meningeal,  etc. 

Diagnosis. — Sporadic  and  epidemic  polioencephalomyelitis  are  con- 
ditioned by  the  same  etiological  factors.  If  an  epidemic  is  in  prog- 
ress, particular  attention  should  be  given  to  all  acute  infections, 
whether  they  show  marked  palsies  or  not,  and  careful  examination 
be  made  of  nerve  tenderness,  hypotonias,  tendon  reflexes,  and  limb 
motility.  Most  cases  develop  either  in  the  guise  of  general  infections 
with  temperature,  or  with  distinct  local  symptoms,  either  in  the  respi- 
ratory or  gastro-intestinal  tracts,  or  in  the  meninges.  Influenza,  poly- 
neuritis, anginas,  bronchitis,  gastro-enteritis,  typhoid  and  epidemic 
cerebrospinal  meningitis  come  into  review.    A  marked  general  hyper- 


316  LESIONS  OF  THE  SPINAL  CORD 

esthesia,  and  a  distinct  pathological  tendency  to  perspiration  is  sig- 
nificant. Leukopenia,  according  to  Miiller,  with  fever  is  also  of 
importance.  Sleepiness,  to  drowsiness  of  the  children,  during  the  day, 
wakefulness  and  fretfulness  at  night,  easy  fatigability,  weakness  of 
the  extremities,  loss  of  muscular  tonus,  especially  in  the  abdominal 
muscles,  with  meteorism  and  loss  of  the  abdominal  reflexes,  point  to 
poliomyelitis.  An  early  lumbar  puncture  will,  resolve  many  of  the 
difficulties.  Influenza  is  separated  with  considerable  difficulty  in  the 
early  stages,  so  much  so  that  certain  observers  (Borstrom)  hold  that 
poliomyelitis  is  nothing  but  a  severe  neural  type  of  influenza. 
Monkey  experimentation  has  disposed  of  this  hypothesis. 

Polyneuritis  also  offers  particular  difficulties.  This  is  rare  in  children, 
apart  from  diphtheritic  neuritis,  and  is  usually  quite  symmetrical  in 
its  development.  The  time  needed  for  development  of  the  palsies  is 
longer,  the  pains  are  more  persistent,  there  are  usually  more  objective 
sensory  disturbances,  particularly  deep  sensibility,  and  early  edemas 
are  more  frequent. 

In  diphtheritic  neuritis,  cardiac  irregularities  are  the  rule,  in  poliomye- 
litis the  exception;  the  palsies  of  the  palate  are  further  characteristic 
in  the  former. 

Pure  neuritic-like  forms  of  poliomyelitis  are  sometimes  present, 
but  there  is  here  more  tendency  for  a  mild  dissociation  syndrome, 
diminution  of  pain  and  temperature  sensibility.  Neuritis  is  more  apt 
to  include  all  the  forms  of  sensibility,  or  gives  a  diminution  in  touch, 
(epicritic)  with  an  increase  in  pain  sensibility.  In  very  small  children 
these  differences  are  difficult  to  bring  out.  Williamson  lays  stress 
upon  the  loss  of  bony  sensibility  in  neuritis,  whereas  in  poliomyelitis  it 
is  rare. 

Further  differentials  from  Wernicke's  polioencephalitis,  superior 
myelitis,  hematomyelia,  myatonia  congenita,  hysteria,  Parrot's  palsy 
in  hereditary  syphilitics,  etc.,  must  be  sought  for  in  monographs. 

In  the  meningitic  forms,  and  in  many  of  the  ordinary  spinal  cases 
the  separation  from  spinal  or  tuberculous  meningitis  is  very  difficult. 
The  lumbar  puncture  here  usually  clears  up  the  diagnosis.  Clinically, 
the  more  marked  mental  symptoms,  the  high  degree  of  stiffness  of 
the  spinal  column,  Kernig's  sign,  and  at  times  papillary  edema,  ear 
complications,  and  herpes,  all  speak  for  meningitis.  In  tuberculous 
meningitis  the  spinal  fluid  reactions  and  the  longer  course  usually 
establishes  the  diagnosis. 

Prognosis. — The  older  teachings  that  the  disease  is  rarely  fatal, 
and  always  shows  persistent  palsies,  must  be  modified  in  both  direc- 
tions. Many  fatal  cases  do  occur,  and  total  recoveries  are  frequent. 
In  certain  epidemics  the  mortality  is  very  high  (42  per  cent.),  in  others 
only  10  per  cenjt.,  counting  only  those  patients  with  evident  palsies. 
In  the  New  York  epidemic  of  1907  the  mortality  was  approximated 
as  5  per  cent. 

If  the  abortive  cases  are  included  the  percentage  falls  markedly. 


ACUTE  POLIOENCEPHALOMYELITIS  317 

The  mortality  is  worse  in  the  older  patients,  as  high  as  50  per  cent, 
in  one  epidemic  (Lindner  and  Malley).  The  period  of  danger  lies 
usually  in  the  fourth  and  fifth  day  of  the  disease.  In  the  second  and 
third  week,  bronchopneumonia  is  a  dangerous  complication. 

As  for  total  recoveries,  they  have  varied  from  10  to  50  per  cent,  in 
the  various  epidemics,  and  are  more  common  in  younger  children  than 
in  those  over  fourteen  years  of  age.     (See  Wickmann.) 

From  the  stand-point  of  electrical  prognosis,  the  older  views  are 
certainly  false.  The  whole  subject  is  in  need  of  entire  revision.  A  total 
loss  of  faradic  excitability  after  a  week  is  no  certain  criterion  of  perma- 
nent palsy,  as  has  been  taught  by  Oppenheim  and  others. 
.  Reparation  takes  place  most  rapidly  in  the  first  six  months,  but  con- 
tinues throughout  a  year  or  more,  and  with  continuous,  rational  treat- 
ment weak  and  paralyzed  muscles  will  continue  to  improve  for  many 
years. 

Treatment. — Prophylaxis,  treatment  of  the  acute  stage,  and  of  the 
chronic  stages  are  to  be  distinguished. 

Prophylaxis. — ^Isolation  and  disinfection  are  as  yet  unprecise  in 
their  application.  The  mode  of  transmission  of  the  virus  is  as  yet 
unknown,  and  whereas  there  is  little  difficulty  in  isolating  the  severely 
attacked,  the  abortive  cases  are  not,  and  rarely  can  be,  properly  regu- 
lated. That  abortive  cases  do  carry  the  disease  seems  definitely  proved. 
The  proper  length  of  time  for  isolation  has  not  been  determined. 
Wickmann  regards  three  weeks  as  sufiScient,  Miiller  eight  weeks.  As 
yet  the  data  do  not  permit  definite  counsels. 

The  virus  in  monkey  poliomyelitis  seems  to  have  been  found  in  the 
nasal  mucous  membranes  and  in  the  feces.  Hydrogen  peroxide,  1 
per  cent,  solution,  and  menthol  solutions  can  be  used  for  the  former, 
formaldehyde  or  carbolic  acid  for  the  latter.  Special  attention  should 
be  directed  toward  the  pocket  handkerchief. 

Rooms  may  be  disinfected  with  formaldehyde.  Preventive  inocula- 
tion, analogous  to  Pasteur  rabies  treatment,  may  become  practicable 
in  time. 

Acute  Stage. — Absolute  rest  in  bed  is  to  be  enforced,  and  if  an 
epidemic  be  in  progress,  children  with  mysterious  diarrheas,  pseudo- 
influenzas,  mild  neuritic  pains,  etc.,  with  fever,  should  be  kept  in  bed, 
even  after  they  appear  to  have  recovered.  The  patients  who  have 
been  sick  for  a  couple  of  days,  and  who  get  up  and  around,  and  are 
then  suddenly  stricken  down,  are  in  reality  very  numerous.  Foresight 
here  cannot  be  overvalued. 

Counter-irritation  to  the  spine,  by  mustard  plaster  or  other  means; 
prompt  catharsis,  first  by  enema  and  then  by  saline  cathartics,  should 
be  employed.  The  motor  restlessness  and  pain  are  best  controlled  by 
analgesics,  and  the  various  salicylate  preparations.  Codein  may  be 
employed,  but  its  inhibitory  action  upon  the  bladder  should  not  be 
overlooked. 

Diaphoresis  by  hot  packs  is  helpful  for  the  pains,  muscular  soreness. 


318  LESIONS  OF  THE  SPINAL  CORD 

and,  possibly,  in  aiding  elimination.  Frequent  treatment  by  free  use  of 
deep,  warm  baths,  102°  to  104°  F.  is  highly  desirable.  The  warm  water 
relieves  the  sense  of  pain  greatly,  and  the  irritability,  and  is  greatly 
appreciated  by  the  patient.  The  baths  can  be  repeated  every  three 
or  four  hours;  the  time  in  the  bath  is  from  ten  to  twenty  minutes. 
On  taking  the  child  from  the  bath,  he  should  be  rolled  in  a  blanket 
and  dried  in  bed.    Continuous  warm  baths  are  worth  trying. 

In  the  later  stages  the  bath  treatment  is  invaluable  for  straightening 
out  the  contracted  limbs  and  aiding  in  active  motion. 

Urotropin  may  be  administered.  It  is  thought  to  reduce  to 
formaldehyde  in  the  cerebrospinal  fluid  in  sufficient  amounts  to 
act  as  an  antiseptic.  The  hypothesis  lacks  definite  experimental 
confirmation. 

Chronic  Stages. — ^After  the  acute  stage  has  passed  there  is  the  long, 
hard  stage,  lasting  for  weeks  or  months  or  years,  of  partial  or  total 
paralysis,  great  weakness,  extreme  tenderness,  nervous  irritability,  and 
muscular  pains,  with  gradually  developing  deformities. 

The  diet  should  be  full  and  regularly  administered.  A  careful 
inventory  should  be  made  of  every  affected  muscle,  both  in  terms  of 
its  functional  capacity,  and  its  electrical  excitability,  the  milliamperes 
necessary  for  bringing  about  contraction  being  carefully  noted.  Full 
measurements  of  the  limbs  should  be  taken.  Careful  note  must  be 
taken  of  the  position  of  the  limb  in  bed,  and  appropriate  support 
given  to  relieve  pain,  avoid  stretching,  and  diminish  contraction. 
Bed-sores,  sore  heels,  elbows,  etc.,  need  very  careful  treatment. 

Chief  reliance  is  now  laid  upon  massage,  passive  motions  and 
resistance  motions.  Here  the  deep,  warm  bath  is  invaluable,  serving 
to  help  the  motions  of  the  limbs,  and  to  straighten  out  the  contractions. 
Movements  in  water  are  more  easily  performed,  and  small  children  can 
play  in  the  deep  tub  for  hours.  Special  exercises  should  be  planned, 
both  for  the  water  and  in  bed.  Special  calisthenics  must  now  be  devel- 
oped, depending  upon  the  muscle  group  involved. 

Particular  attention  must  be  directed  to  the  mental  life  of  the  child. 
He  is  apt  to  be  morose,  reticent,  shy,  and  resentful,  becomes  very 
sensitive  if  his  deformity  be  marked,  and  selfishness  and  exactingness 
develop  easily  if  pampered  because  of  his  weakness. 

In  the  later  stages,  and  for  those  old  enough,  swimming  is  the  best 
exercise. 

The  correction  of  the  deformities  is  a  matter  for  the  orthopedic 
surgeon.  Mechanical  aid  should  be  given  as  soon  as  possible,  if  it 
carries  out  a  progressive  therapeutic  principle.  Operative  procedures 
should  be  conservatively  considered,  and  not  used  too  early.  These 
patients  make  wonderful  recoveries  unaided,  or  by  the  persistent  use 
of  massage  and  mechanotherapy.  Many  operative  procedures  are, 
however,  imperative.  Nerve  splicing,  and  tendon  splicing  have  their 
special  indications. 

Electrical  therapy  is  of  use  only  as  a  bridge  to  gap  the  period  after 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES  319 

the  first  onset  until  such  time  as  definite  willed  movements  can  be 
started. 

Drug  therapy  is  of  purely  symptomatic  value.  Iron,  strychnine, 
calcium  are  the  most  useful  remedies,  and  meet  special  indications. 

PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES. 

The  spinal  •  motor  neuron  is  differentiated  into  three  structures, 
termed  the  anterior  horn  cell,  the  motor  nerve  fiber,  and  the  muscle 
plate.  It  would  be  a  great  advance  toward  the  simplification  of  the 
vexed  questions  regarding  this  group  if  a  clinical  division  could  be 
made,  in  strict  accordance  with  the  pathological  alterations  of  these 
structures.  To  a  certain  extent  this  is  justified.  For  categorical  pur- 
poses then  one  can  distinguish:^ 

1.  The  progressive  nuclear  atrophies,  or  myelogenous  or  myelo- 
pathic types:  Etiology:  Heredity,  trauma,  cold,  toxins,  infections. 
Anatomy:  Primary  degeneration  of  the  anterior  horn  cells,  with 
secondar}^  fiber  or  muscle  changes.  Forms:  Infantile,  Hoffmann- 
Werdnig;  ad^lt,  Aran-Duchenne;  mixed  forms. 

2.  The  neural,  neuritic,  or  spinal  neuritic  atrophies:  Etiology: 
Heredity.  Anatomy:  Primary  degeneration  of  the  motor  nerve  fibers 
with  secondary  ganglion,  cord,  and  muscle  changes.  Forms:  Peron- 
eal-arm  type  (Charcot-Marie-Tooth) ;  tabetic  type  (Dejerine-Sottas) ; 
peroneal  type  and  arm  type  (Sainton  and  Haenel) . 

3.  The  muscular  dystrophies  or  myogenic  types.  Etiology:  Heredity. 
Anatomy:  Primary  changes  in  muscles.  Forms:  Hereditary  (Leyden- 
Mobius);  juvenile  pseudohypertrophy  (Landouzy-Dejerine) ;  and- 
mixed  forms  (Erb-Zimmerlin). 

The  separation  of  these  forms  has  occupied  the  attention  of  neurol- 
ogists since  about  1849  when  Duchenne  first  attacked  the  problem  of 
dismemberment  cf  this  large  group  with  muscular  wasting. 

Clinicians  for  many  centuries  had  described  progressive  muscular 
wasting.  Pictures  and  images  in  stone  and  wood  of  the  muscular 
atrophies  and  muscular  dystrophies  dating  from  the  fifteenth,  sixteenth 
and  seventeenth  centuries  are  in  existence.  Van  Sweiten,  Abercrombie, 
and  others  gave  general  descriptions.  This  group  was  first  broken 
into  by  Duchenne,  in  1849,  by  the  loose  description  of  a  special  type, 
which  a  year  later  Aran  (1850)  supplemented.  Cruveilhier,  in  1853, 
and  Luys,  in  1860,  sharpened  the  picture  somewhat  by  their  demonstra- 
tion of  the  exclusive  implication  of  the  anterior  horns.  In  1865 
Charcot  eliminated  the  amyotrophic  lateral  scleroses  from  the  group. 
Duchenne  himself  had,  in  1853,  also  set  aside  the  pseudohypertrophies, 
the  muscular  features  and  varieties  of  which  w^ere  later  demonstrated 
by  Eulenberg  (1866),  Charcot,  Leyden,  and  Dejerine.  Wachsmuth, 
in  1864,  recognized  the  bulbar  forms.     Finally,  Duchenne  also  called 

1  Kiigelgen,  Beitrag.  zur  neuralen  progressive!!  Muskeltrophien,  Arch.  f.  Psych.,  1909, 
xlv,  944. 


320  LESIONS  OF   THE  SPINAL  CORD 

attention  to  the  presence  of  sensory  anomalies  in  certain  of  his  1853 
studies  on  the  atrophies;  these  were  for  the  most  part  syringomyeHas, 
which  Kahler  and  Schultze  separated  definitely  in  1882.  Another 
small  group  were  the  neuritic  atrophies,  first  clearly  recognized  by 
Dumesnil  (1864).  Thus  it  took  thirty  years  for  the  sorting  out  of 
this  medley  of  muscular  atrophies;  the  groups  as  they  are  at  present 
recognized,  at  least  two  of  which,  syringomyelia  and  amyotrophic 
lateral  sclerosis,  being  entirely  set  outside  of  the  muscular  atrophies 
per  se.  One  result  was  that  the  original  disorder  of  Aran-Duchenne 
was  so  much  reduced  that  finally  Marie,  in  1894,  tried  to  sweep  it 
away  entirely,  but  his  iconoclasm  has  not  been  justified. 

Group  1.    The  Progressive  Nuclear  Atrophies:  (1)  Spinal,  (2)  Bulbar, 
and  (3)  Mesencephalic  Forms. 

Three  spinal  main  forms  are  here  to  be  recognized,  (a)  Chronic 
poliomyelitis  per  se,  with  lesions  limited  primarily  to  the  motor  ganglion 
cells;  (b)  Aran-Duchenne's  disease,  progressive  spinal  muscular  atrophy 
and  (c)  infantile  hereditary — Werdnig-Hoffmann. 

The  former  two  forms  are  much  alike,  and  many  authors  maintain 
their  identity.  They  are  here  considered  separately,  since  such  a 
course  seems  justified  clinically  if  not  pathologically.^ 

(a)  Chronic  Poliomyelitis. — Here  are  grouped  those  rare  subacute 

or  chronic  muscular  atrophies  usually  occurring  in  the  late  years 

of  life,  in  which  certain  toxic  agents  seem  to  account  for  the  disorder. 

Such  toxic  factors  are  lead,  diabetes,  syphilis,  and  other  unknown 

-toxemias.     Heredity  is  sometimes  present. 

The  antomical  lesions  are  a  progressive  destruction  of  the  motor 
ganglion  cells,  with  chronic  vascular  alterations. 

Symptoms. — There  is  a  progressively  developing  flaccid  paralysis, 
with  antecedent  weakness,  without  sensory  or  trophic  signs,  and 
diminution  or  loss  of  tendon  reflexes.  The  nerve  trunks  are  not 
tender  or  swollen.  Fibrillary  twitches  appear,  the  myotatic  irritability 
is  increased,  reaction  of  degeneration  is  present.  There  are  no  disturb- 
ances of  the  rectal,  vesical,  or  sexual  functions,  and  the  medullary 
nuclei  are  rarely  involved. 

The  localization  may  vary;  the  legs  or  arms  first  showing  weakness 
or  atrophy,  sometimes  one  arm  or  both,  one  arm  and  one  leg,  or  both 
legs.  The  atrophy  and  palsies  take  place  usually  slowly,  with  at  times 
periods  of  more  rapid  development,  and  also  stationary  periods  during 
which  no  advance  is  made  for  years,  or  the  patient  slowly  recovers. 

The  distribution  of  the  atrophic  muscles  is  characteristic.  Certain 
muscle  groups  are  spared.  The  arrangement  is  segmental,  not  radicu- 
lar. The  triceps  may  escape  with  all  the  other  muscles  of  the  arm 
attacked,  or  the  flexors  of  the  fingers  may  be  intact.     In  the  legs  the 

1  Marburg,  Handbuch.  d.  Neurologie,  1911,  vol.  ii,  p.  280,  for  complete  literature; 
Lovegreen,  Zur  Kenntnis  der  Pol.  acuta  und  chronica,  Karger,  1904. 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES 


321 


tibialis  anticus  may  stand  out  unimpaired.  The  course  is  usually 
very  chronic,  lasting  years.  Subacute  onsets  are  more  favorable. 
Some  patients  recover,  especially  those  with  suspicion  of  a  radiculitis 
or  a  neuritis.  In  the  progressive  cases  the  patients  at  times  develop 
bulbar  symptoms,  and  die  of  aspiration  pneumonia,  or  weakness. 

Treatment. — Rest,  hydrotherapy,  gentle  massage,  electricity,  over- 
feeding are  the  chief  indications.  Syphilitic  cases  need  specific 
therapy. 

(b)  Aran-Duchenne  Type. — Progressive  Muscular  Atrophy. — Etiology. 
—  Here  heredity  may  play  a  part  in  the  development  of  a  familial  form, 
seen  in  infancy,  and  also  in  adults  (Bernhardt).  It  may  follow  acute 
poliomyelitis.     Other  factors,   as  toxemias,   trauma,   cold   and  wet. 


Fig.  158. — Chronic  poliomyelitis  showing  atrophy  of  hands. 

overexertion,  are  not  definitely  established.  Occupation  atrophies 
are  at  times  incorrectly  included  here.  Pathologically  there  is  degen- 
eration of  the  motor  ganglion  cells  and  fibers,  with  some  secondary 
degenerations  in  the  anterior  lateral  columns. 

It  is  a  rare  disorder.  The  disease  begins  very  slowly,  the  upper 
extremities  are  attacked,  and  rarely  the  lower.  The  smaller  muscles 
of  the  hand  are  initially  affected.  Weakness,  atrophy,  and  flaccid 
paralysis  is  the  sequence.  Fibrillary  twitchings  accompany  the 
atrophy  and  there  is  reaction  of  degeneration  in  the  muscles  involved. 
Sensory  and  trophic  disturbances  are  lacking  and  the  tendon  reflexes 
are  diminished  or  lost.  Early  over-excitability  of  the  tendon  reflexes 
may  be  noted  in  both  of  these  forms. 
21 


322 


LESIONS  OF  THE  SPINAL  CORD 


Oppenheim  has  called  attention  to  the  tendency  for  these  atrophies 
to  jump  from  one  muscle  group  to  another.  The  atrophy  is  very 
gradual;  the  disease  advances  slowly  for  years.  Remissions  occur, 
but  recoveries  are  very  unusual. 

The  distribution  of  the  affected  muscles  varies  considerably.  Death 
usually  results  from  paralysis  of  the  muscles  of  respiration. 

Diagnosis. — The  separation  of  these  two  forms  is  at  times  impossible. 
There  is  a  tendency  for  the  former  to  advance  more  rapidly,  to  involve 
the  lower  limbs,  to  advance  to  the  medulla,  to  show  longer  remissions, 


Fig.  159. — Primary  nuclear  atrophy. 
(G.  M.  Hammond.) 


Fig.  160. — Primary  nuclear  atrophy. 
(G.  M.  Hammond.) 


and  to  recover.  Further,  there  is  a  greater  tendency  to  segmental 
distribution  in  the  muscle  groups  affected  in  the  former  type,  with 
marked  loss  of  power  followed  by  atrophy.  In  the  latter  type  the 
atrophy  seems  to  precede  the  palsies,  and  the  patients  are  often  very 
adept  with  their  residual  muscles. 

The  sensory  disturbances  of  occupation  atrophies,  of  neuritis,  of 
syringomyelia  should  exclude  these,  while  the  increased  tendon  reflexes 
of  amyotrophic  lateral  sclerosis  eliminates  that  disorder.  Radiculitis 
needs  separation,     Here  the  atrophies  are  apt  to  be  unilateral,  and  are 


PRIMARY  PROGRESSIVE  MtlSCULAR  ATROPHIES  323 

radicular.    There  is  a  low-grade  neuritis,  and  the  Dejerine-Klumpke 
syndrome  appears  in  the  cervical  type. 

Treatment. — This  may  be  treated  as  the  preceding  affection,  but 
such  therapy  seems  of  little  avail.  Strychnin,  arsenic,  and  fats  are 
indicated.  Newer  ideas  must  be  gained  if  any  effective  control  of 
this  disorder  is  to  be  hoped  for;  the  older  methods  are  useless. 

(c)  Infantile  Hereditary  Forms  (Werdnig^-Hoff mann^) . — ^These  are 
rare  hereditary  types,  beginning  in  infancy,  first  described  in  1891. 
The  disease  begins  gradually,  usually  in  the  pelvic  girdle  and  thighs 
(ileopsoas,  quadriceps  femoris).  Later  the  back,  neck,  and  shoulder 
girdle  is  involved.  Finally  the  distal  extremities  are  involved.  The 
intercostals  and  diaphragm  are  often  affected.  Occasionally  the 
bulbar  muscles  atrophy.  The  atrophies  are  usually  symmetrical, 
often  associated  with  apparent  hypertrophy  (adiposis). 

There  is  gradually  increasing  paresis  and  loss  of  skin  and  tendon 
reflexes.  Fibrillary  twitching  seems  absent  and  a  peculiar  trembling 
of  the  fingers  is  described.  Scolioses,  talipes,  and  other  contracture 
states  develop.  These  little  patients  often  acquire  unique  modes  of 
locomotion.  Faradic  currents  are  borne  better  than  galvanic  currents, 
but  reaction  to  both  is  diminished  or  lost. 

The  course  is  usually  progressive,  rarely  stationary."*  Mental 
reduction  is  not  usual. 

(2)  Bulbo-pontine  Types.  Chronic  Progressive  Bulbar  Palsies. — In 
these  forms  the  progressive  atrophy  is  limited  to  the  muscles  of  the 
face,  tongue,  palate,  and  larynx. 

Etiology. — Nothing  is  known  of  the  causation  factors.  Certain 
toxic  factors — lead,  syphilis — have  seemed  to  play  a  role  at  times; 
constantly  recurring  electrical  shocks  was  a  factor  in  one  patient 
personally  observed;  the  majority  give  no  clue  as  to  etiology.  A  few 
cases  are  observed  in  childhood,  still  fewer  in  adult  manhood;  the 
majority  occur  after  thirty-five  years. 

Symptoms. — There  is  a  slow  progressive  weakness  of  the  tongue,  and 
muscles  of  the  cheeks  and  lips,  followed  by  fibrillary  twitchings  and 
slowly  progressive  atrophy.  Speaking  becomes  fatiguing,  and  slight 
changes  in  the  voice  become  apparent.  In  eating  the  patients  find 
they  must  use  their  fingers  in  dislodging  food  from  behind  the  teeth  in 
the  cheek.  Gradually  increasing  difficulty  in  swallowing  is  observed, 
and  difficulties  in  breathing  appear.  Finally  the  speech  becomes 
lalling,  dysarthric,  the  lingual  letters  d,  t,  I,  r,  n,  s,  are  first  slurred 
over,  then  the  labial  letters  h,  p,  f,  m,  w,  o,  v,  e.  The  laryngeal  weakness 
causes  hoarseness,  monotony,  and  finally  aphonia.  The  pharyngeal 
muscles  and  those  of  the  tongue  do  not  act  in  concert,  and  liquid  food 
regurgitates  through  the  nose. 

1  Archiv  f .  Psychiatrie,  22,  26. 

^  Deutsche  Zeitschrift  f.  Nervenheilkunde,  3,  10,  18. 

5  Late  literature  Batten.  Lancet,  June  3,  1911;  Senator,  Charite  Annalen,  1902;  Lange, 
Deutsche  Zeitschrift  f.  Nervenheilkunde,  1910,  40. 


324  LESIONS  OF  THE  SPINAL  CORD 

The  face  becomes  atrophied  below,  the  Hps  thin  and  folded.  The 
patients  cannot  protrude  the  lips,  nor  whistle.  The  temporals  and 
masseters  also  become  affected,  and  the  movements  of  the  jaw  become 
impossible.  The  masseter,  pharyngeal,  and  vomiting  reflexes  are  absent. 
The  sensibility  is  intact.  The  upper  face  region,  including  the  levator 
palpebrse,  is  rarely  affected. 

Vasomotor  disturbances  are  occasionally  observed.  Vagus  involve- 
ment causes  cardiac  irregularity. 

The  usual  progression  is  from  the  tongue  to  the  lips,  then  to  the  other 
facial  muscles,  and  finally  to  the  fifth,  and  hypoglossal  and  vagus 
muscles.  An  apparent  increase  in  saliva,  an  annoying  symptom,  is 
largely  dependent  upon  the  inability  to  swallow. 

The  usual  course  extends  over  four  or  five  years,  death  taking  place 
most  frequently  from  pneumonia  or  from  inanition. 

Pathology. — Here  one  finds  changes  in  the  medullary  nuclei,  precisely 
analogous  to  those  found  in  progressive  spinal  nuclear  atrophy.  The 
corticobulbar  tract  may  be  involved  somewhat,  but  rarely  to  the  degree 
found  in  amyotrophic  lateral  sclerosis,  yet  intermediary  forms  are  to 
be  expected. 

Diagnosis. — Unusual  types  of  gliosis,  of  multiple  sclerosis,  tumors, 
gummata,  arteriosclerosis,  general  paresis  may  cause  somewhat 
similar  pictures  at  first,  but  these  also  soon  show  other  symptoms, 
indicating  that  the  lesion  is  not  confined  to  the  nuclear  structures. 
Pseudobulbar  palsy  shows  palsied  muscles,  still  electrically  reflexly 
excitable,  without  atrophies.  Myasthenia  gravis  shows  the  typical 
electrical  myasthenic  reaction. 

Prognosis. — Absolutely  bad,  save  in  syphilitic  cases. 

Treatment. — Thus  far  none  is  known.  One  should  always  be  on  the 
lookout  for  the  other  causes  of  the  syndrome,  however,  in  the  initial 
stages.  Here  a  nihilistic  therapy  will  be  recognized  too  late  for 
recovery. 

Galvanization  has  seemed  to  help  the  swallowing  of  some  patients. 
Atropin,  hyoscyamus,  etc.,  or  other  related  drugs  can  be  used  to 
control  the  salivation,  and  the  pains  and  discomforts  of  coughing, 
dyspnea,  etc.,  relieved  by  narcotics.  Specific  therapy  is  valuable  in 
the  Wassermann  positive  cases. 

3.  Pontomesencephalic  Forms. — Chronic  Progressive  Ophthalmo- 
plegia.— Chronic  progressive  ophthalmoplegias  as  a  part  of  an  amyo- 
trophic lateral  sclerosis,  of  tabes,  of  general  paresis,  multiple  sclerosis, 
tumor  of  corpora  quadrigemina,  or  other  organic  disease  are  not  rare. 
As  pure,  chronic  nuclear  affections  they  are  infrequent.  Uthoff 
credits  them  as  high  as  14  per  cent,  of  the  chronic  ophthalmoplegias. 

There  is  beginning  weakness  of  the  eye  muscles,  usually  the  external 
rectus,  with  slight  internal  strabismus,  frequently  worse  at  night. 
Diplopia  is  not  usually  present,  because  of  the  gradual  development  of 
the  disorder,  and  its  symmetry.  In  hereditary  types,  in  infants, 
binocular  vision  may  not  have  developed. 


NEURITIC  ATROPHIES  325 

The  internal  eye  muscles  are  usually  intact,  although  occasionally 
irregular  pupils  occur;  light-immobile  pupils  are  not  present  although 
loss  of  accommodation  movements  have  occurred.  Ptosis  is  not 
infrequent;  usually  worse  on  one  side. 

In  a  completely  developed  case  the  Hutchinson  face  develops 
bilateral  ptosis  with  inability  to  move  the  eyeballs. 

The  disorder  is  usually  progressive.  Starr  has  described  a  stationary 
condition,  and  some  patients  recover  entirely. 

Treatment. — When  a  Wassermann  test  has  revealed  a  positive 
reaction,  mercury  and  iodides  are  indicated;  otherwise  general  tonics, 
strychnin,  iron,  arsenic,  and  general  dietetic  treatment  directed  to 
the  reduction  of  arteriosclerotic  changes. 

Group  2.     The  Neural,  Neuritic,  or  Spinal  Neuritic  Atrophies. 

Hard  and  fast  lines  cannot  as  yet  be  drawn  between  the  neuritic 
atrophies  from  certain  nuclear  atrophies  on  the  one  hand,  nor  certain 
dystrophies  on  the  other.  Jendrassik,  in  a  recent  monograph  (1911), 
speaks  of  them  as  "  dystrophy  forms  with  degeneration  of  the  peripheral 
nerves." 

Many  forms  of  neural  atrophy  have  been  described.  The  most 
characteristic  are:  (1)  the  peroneal,  forearm  type — Charcot-Marie- 
Tooth;  (2)  the  tabetic,  or  hypertrophic  interstitial  neuritic  type — 
Dejerine-Sottas,  Marie's  familial  form;  (3)  the  peroneal  type — Sainton; 
(4)  the  arm  type— Haenel. 

1.  Peroneal-forearm  Type. — Charcot,  Marie  and  Tooth  described 
this  form  which  is  characterized  by  muscular  wasting  in  the  distal 
parts  of  the  extremities,  from  knee  and  elbow  outward.^ 

Etiology. — Eichorst  found  thirteen  cases  in  six  generations  and 
Haenel  thirty-two  in  four,  while  others  have  found  only  one. 

Symptoms. — The  feet  are  usually  first  involved.  There  is  early 
equinus  position  from  loss  of  power  of  extension  of  the  great  toe. 
Then  the  leg,  from  the  knee  down,  thins  and  atrophies.  The  gait 
thereby  becomes  either  widespread  or  steppage.  A  similar  process 
begins  in  the  forearms,  usually  advancing  from  the  muscles  of  the  hand. 
Monkey  hand,  or  main  en  griffe  develops.  Rarely  the  upper  extremity 
atrophy  precedes.  The  shoulder,  arm,  neck,  back,  hips,  and  thigh 
muscles  remain  intact. 

Sensory  changes  are  usually  present,  and  slight  pains,  intermittent 
and  lancinating  in  character,  coming  and  going,  hyperesthesia,  par- 
esthesia, hypesthesia,  especially  to  cold,  are  present.  Painful  nerve 
trunks  are  occasionally  found.  Vasomotor  disturbances  are  frequent. 
Cold  marble-like  skin  with  increased  secretion  is  observed. 

The  knee-jerks  are  often  initially  increased;  later  they  are  diminished 

1  1881,  Rev.  de  Med.,  1886;  Brain,  x,  243;  Spiller,  Journal  of  Nervous  and  Mental 
Disease,  1907. 


326 


LESIONS  OF  THE  SPINAL  CORD 


or  absent.  The  Achilles  reflex  is  usually  absent,  as  is  the  radius- 
periosteal  reflex. 

Reaction  of  degeneration  in  the  regions  afi^ected  is  the  rule.  Fibril- 
lary contractures  and  muscle  unrest  are  constant,  often  persisting 
during  sleep.     Tremors  and  choreic-like  movements  are  not  uncommon. 

Course. — The  disease  usually  begins  in  childhood,  advances  slowly, 
usually  progressively,  sometimes  halting  for  long  periods. 

Pathology. — Since  Hoffmann's  description  (1889)  of  a  neuritis  in 
the  distal  peripheral  nerves,  this  disorder  has  passed  as  a  mixed  neu- 


FiG.   161. — The  neuritic  form  of  muscular  atrophy.      (Spiller.) 

ritic  atrophy,  but  later  Siemerling,  Gierlich,^  Kugelgen,^  Spiller^  and 
others  have  shown  changes,  not  only  in  the  peripheral  nerves  and 
muscles,  but  that  there  were  extensive  changes  in  the  other  parts  of 
the  nervous  system,  in  the  eighteen  to  twenty  cases  thus  far  autopsied 
(1910).  These  show  parenchymatous  and  fatty  degeneration  of  the 
muscles,  ascending  degeneration  of  the  peripheral  motor  nerves, 
especially  intermuscular  branches,  with  chronic  interstitial  .neuritis, 


1  Arch.  f.  Psychiatrie,  1909. 


2  Ibid. 


3  Loo.  cit. 


NEURITIC  ATROPHIES 


327 


degeneration  of  spinal  ganglia,  atrophy  and  loss  of  anterior  horn  cells, 
sclerosis  of  columns  of  Goll,  and  Burdach,  with  changes  in  lateral 
columns  and  posterior  roots. 

2.  Tabetic  Type  (Dejerine-Sottas^). — This  is  apparently  an  hereditary 
form,  the  symptoms  beginning  in  childhood.  The  muscular  changes 
are  similar  to  those  seen  in  the  Charcot-Marie  forms,  but  the  sensory 
nervous  system  is  much  more  markedly  involved.  There  are  present 
pupillary  changes — myosis,  or  even  Argyll-Robertson,  beginning  or 


Fig.  162. — The  neuritic  form  of  muscular  atrophy.     (SpUler.) 

complete  Romberg,  nystagmus,  and  ataxia.  Pain  is  present.  The 
peripheral  nerves  are  enlarged  and  palpable.  Reaction  of  degeneration 
was  stated  to  be  absent  by  Dejerine  and  Sottas. 

It  may  easily  be  confounded  with  a  juvenile  tabes.  Dejerine 
maintains  that  it  has  no  relation  to  the  Charcot-Marie  atrophies. 

Other  forms  are  described  in  which  the  atrophies  are  limited  to  the 


1  Arch.  d.  Neuro;  II  Ser.,  vol.  xvii,  No.  91. 
1906. 


Rev.  de  Med.,  1897;  Rev.  Neur.,  1902, 


828  LESIONS  OF  THE  SPINAL  CORD 

lower  extremity  (Sainton),  to  the  upper  extremity  (Haenel),  to  the 
femorotibial  region  (Eichorst).     The  transition  types  are  many. 

Diagnosis  .^ — A  complete  separation  of  all  the  forms  is  not  possible 
in  the  present  state  of  our  knowledge.  The  Wassermann  reaction  and 
cerebrospinal  fluid  examination  will  probably  throw  considerable 
light  on  the  hypertrophic  neuritic  types  of  Dejerine,  and  may  remove 
them  from  this  group  entirely;  also  cases  described  by  Marie  under  a 
similar  name. 

For  the  classical  cases  of  neuritic  muscular  atrophy  there  is  little 
ground  for  confusion,  but  the  numerous  aberrant  forms  introduce 
difficulties. 

The  separation  from  the  dystrophies  is  usually  made  on  the  ground 
of  pseudohypertrophy  and  the  absence  of  reaction  of  degeneration  in 
these  cases.     Certain  transition  forms  are  undifferentiable. 

Chronic  polyneuritis  is  rarely  hereditary  (unless  one  follows  Oppen- 
heim  in  classing  the  neuritis  atrophies  here  under  consideration  as 
hereditary  chronic  multiple  neuritis).  In  polyneuritis  there  is  rarely 
a  club-foot,  the  progress  is  more  rapid,  and  recovery  is  apt  to  take 
place.  From  tabes,  only  Dejerine's  type  can  cause  confusion.  The 
age  and  heredity  are  the  chief  features.  Newer  studies  on  comple- 
ment fixation  and  spinal  fluid  are  wanting. 

In  amyotrophic  lateral  sclerosis  the  increased  reflexes,  the  extension 
to  the  bulbar  nuclei,  the  spasticity,  and  rapid  course  are  characteristic. 
Multiple  sclerosis,  syringomyelia,  chronic  poliomyelitis,  myotonia, 
and  hereditary  ataxia  occasionally  call  for  differentiation. 

Prognosis. — Usually  poor,  but  the  disease  is  very  chronic,  and 
patients  die  after  twenty  to  thirty  years  of  intercurrent  disorders. 
Sometimes  stationary  periods  are  met  with. 

Therapy. — Electricity,  baths,  massage,  internal  secretions,  general 
tonics,  and  orthopedics  are  useful.     A  few  cures  are  known. 

Group  3.    The  Muscular  Dystrophies  or  Myopathies. 

This  very  large  and  extremely  motley  group  has  also  been  built 
up  of  a  variety  of  forms  since  Duchenne,  in  1849,^  first  described  the 
fatty  pseudohypertrophies,  and  later,  in  1868,  spoke  of  them  as 
myoscleroses.  Leyden^  (1876),  Mobius^  (1879)  described  certain 
hereditary  forms,  while  Erb*  in  1883  first  brought  some  order  into  the 
confusion  of  the  atrophies  and  dystrophies  by  showing  that  in  certain 
forms  this  lesion  was  predominantly  muscular  and  not  nervous. 
He  made  the  first  practical  synthesis.  Landouzy  and  Dejerine^  in 
1884  described  their  well  known  form,  and  separated  it  from  Erb's 
juvenile  type.     Since  this  time  the  group  has  been  better  unified, 

1  Union  Med.,  1853.  *  Klinik  de  R.  II,  1876. 

3  Volkmann's  Klinik,  No.  171.  .  *  Neurol.  Ctbl.,  1883,  p.  452. 

^  Comptes  rendus,  1884,  p.  53. 


MUSCULAR  DYSTROPHIES  329 

its  limits  better  recognized,  and  the  various  forms  within  it  more 
thoroughly  studied.  ^ 

The  myopathies  make  a  fairly  consistent  group,  although  the  forms 
may  not  resemble  one  another  clinically  at  different  periods  of  their 
development,  yet  they  have  a  number  of  common  factors. 

Heredity  is  a  common  feature;  they  usually  occur  at  an  early  age; 
the  muscles  become  weak  gradually  and  atrophy  in  a  peculiar  manner, 
in  that  true  hypertrophied  fibers  are  mingled  with  atrophied  fibers. 
The  muscular  atrophy  may  involve  all  of  the  muscles  equally,  or  may 
be  irregularly  distributed  both  as  to  the  body  in  general  or  within  the 
muscle  itself.  Reaction  of  degeneration  and  fibrillary  contractions 
are  usually  wanting,  although  a  gradual  loss  of  electrical  excitability 
goes  on  coincidently  with  the  atrophy. 

Certain  muscles,  pectoralis  major,  rhomboid,  serratus  magnus, 
are  oftenest  the  seat  of  early  atrophy.  These  are  also  characterized 
as  congenital  aplasias  (Bing).  The  muscle  electrical  reaction  curve  is 
striking. 

The  tendon  reflexes  gradually  disappear,  but  the  Achilles  is  apt  to 
persist,  or  occasionally  be  increased,  especially  with  much  pseudo- 
hypertrophy. Sensory  disturbances  are  usually  absent,  likewise 
bladder  and  visceral  disturbances.  Pseudo-contractures  with  limita- 
tion of  movement  is  frequent,  causing  peculiar  positions.  The  patients 
hop  like  frogs.  Bony  dystrophies  are  also  frequent,  mostly  showing 
in  thinning  of  the  long  bones,  with  cranial  deformities,  deformed 
hands,  short  hands  and  short  feet. 

Pathology. — Erb  based  his  synthesis  upon  the  changes  he  found 
in  the  muscles,  but  at  the  same  time  was  inclined  to  attribute  them 
to  lesions  in  the  trophic  centers.  These  muscle  changes  consist 
in  the  main  in  hypertrophy  and  atrophy,  and  splitting  of  the  muscle 
fibers,  proliferation  of  the  nuclei,  new  connective  tissue  proliferation 
with  hyperplasia  of  the  vascular  tissues  and  fatty  deposition.  Macro- 
scopically  the  muscles  have  lost  their  normal  color,  varying  from  pale 
pink  to  dark  red.  In  places  where  the  muscle  substance  has  entirely 
disappeared  white  connective  tissue  is  apparent. 

The  peripheral  nervous  system  is  usually  negative.  The  muscle 
plates  are  frequently  missing. 

Loss  of  cells  in  the  ventral  horns  has  been  described  by  Holmes^  and 
others.  Whether  these  are  primary  or  secondary  cannot  yet  be 
determined. 

The  change  in  the  bones  is  in  the  nature  of  a  rarefaction. 

Our  knowledge  of  the  trophic  cells  of  the  cord  is  still  too  incomplete 
to  overthrow  Erb's  original  conclusion,  although  the  evidence  seems  to 
point  to  this  group  as  one  of  muscular  origin. 


1  Batten,  The  Myopathies  or  Muscular  Dystrophies,  Quart.  Jour.  Med.,  April,  1910. 
Lorenz.  Krankheiten  d.  Muskeln,  1904;  Jendrassik,  Handbuch  d.  Neurol.,  1911. 

2  Rev.  Neur.  and  Psych.,  1906,  vi,  p.  136. 


330 


LESIONS  OF  THE  SPINAL  CORD 


Forms. — The  forms  are  many  and  show  considerable  intermingling. 
They  are : 

1.  Pseudohypertrophic  (Duchenne). 

2.  Juvenile  (Erb). 


:j 


Fig.  163. — Pseudohypertrophic 
myopathy.  Early  stage.  (Jen- 
drassik.) 


Fig.     164.  —  Pseudohypertrophic    myopathy. 
Stage  of  atrophy.     (See  163.)      (Jendrassik.) 


3.  Facio-scapulo-humeral  (Landouzy-Dejerine). 

4.  Atrophic  myatonia  congenita  (Oppenheim). 

5.  Myatonic  atrophica. 

6.  Distal  (Gowers). 

7.  Mixed  and  transitional  forms. 


MUSCULAR  DYSTROPHIES 


331 


1.  Pseudohyperiroj^hic  Type  (Duchenne,  1849). — Semmola,  in 
1834,  and  Costa  and  Gioja,  in  1836,  antedated  Duchenne  in  describing 
these  cases,  but  pictures  of  earlier  centuries  give  evidence  of  its  pres- 
ence. It  is  the  type  most  frequently  observed.  It  is  more  common 
in  males  (3  to  1),  and  usually  begins  during  childhood.  An  hereditary 
form  is  very  frequent.  The  parents  first  notice  a  certain  clumsiness 
in  the  gait  of  the  children,  then  the  position  of  the  body  is  peculiar. 


Fig.  165. — Pseudohypertrophic  myopathy.    Later  stage  of  atrophy.    Compare  163,  164. 

(Jendrassik.) 

the  head  being  bent  forward,  and  the  cervical  vertebrae  are  particularly 
prominent.  There  is  an  early  beginning  lumbar  lordosis.  The  patient 
waddles,  then  commences  to  find  it  hard  to  go  up  stairs — often  trips  and 
falls.  On  rising  from  a  recumbent  position  the  arms  are  called  in  to 
aid,  and  the  mode  of  rising  is  unique.  The  patient  climbs  up  his 
legs  as  it  were  with  his  arms.  In  the  final  stages  the  patient  is  unable 
to  raise  himself  at  all. 


332 


LESIONS  OF  THE  SPINAL  CORD 


The  shoulder-blades  are  freely  movable  and  rise  with  the  rise  of  the 
arms.    Atrophies  are  apparent. 

The  gait  varies  somewhat,  according  to  the  muscles  chiefly  involved. 
It  is  often  wabbly,  like  a  pregnant  woman;  at  times  it  has  a  high  step 


Fig.    166. — Pseudohypertrophic   myopathy.      Later   stage.      Compare    163,    164,    165. 

(Jendrassik.) 

character;  again  the  patient  walks  on  his  toes.  The  lower  limbs  often 
show  marked  hypertrophy  in  the  early  stages,  the  calves  are  plump 
and  firm. 


Fig.  167. — Pseudohypertrophic  myopathy.    Later  stage.    Compare  163,  164,  165,  166. 

(Jendrassik.) 


The  atrophy  advances  unequally.  The  muscles  most  affected  in 
the  lower  extremities  are  the  psoas,  glutei,  quadriceps,  sartorius, 
adductors,  gastrocnemius  and  soleus;  in  the  trunk,  the  rectus 
abdominis,  latissimus  dorsi,  erector  spinse,  rhomboidei,  infraspinatus, 


MUSCULAR  DYSTROPHIES 


333 


serratus,  trapezius,  and  pectoralis  major,  while  in  the  upper  extremities 
the  muscles  mostly  implicated  are  the  deltoid,  biceps,  brachialis,  and 
brachioradialis. 

In  advancing  cases  all  of  the  muscles  go,  save  perhaps  those  of  the 
face.     In  rare  cases  the  face  is  involved   (myopathic  facies),   and 


Fig.  168. — Pseudohypertrophic  myopathy. 
(G.  Hammond.) 


Fig.  169. — Pseudohypertrophic 
myopathy.     (G.  Hammond.) 


in  a  few  cases  bulbar  muscles  are  diseased.  In  the  less  advanced 
cases  many  of  the  distal  muscles  can  be  utilized. 

The  patients  usually  die  of  intercurrent  disorders  after  many  years 
of  illness. 

2.  Juvenile  Form  (Erb). — This  usually  develops  about  the  age  of 
puberty,  with  weakness  and  atrophy  in  the  shoulder  girdle.     The 


334 


LESIONS  OF  THE  SPINAL  CORD 


deltoid  may  show  hypertrophies.  The  arm  is  usually  thinner  and  more 
atrophic  than  the  forearm,  and  typical  winged  scapulae  develop. 
In  walking  the  patients  not  infrequently  bend  forward,  from  weakness 
of  the  trunk,  and  support  the  back  by  holding  the  thighs.  Pseudo- 
hypertrophy of  the  calves  is  not  uncommon. 

3.  Facio-scapulo-humoral  Type  (Landouzy-Dejerine).  —  Here  the 
facial  atrophies  usually  develop  early,  particularly  the  orbicularis 
oris.  The  sphinx-like  face  develops,  the  patients  are  unable  to  whistle, 
tapir  mouth  is  often  present,  and  their  smile  is  distorted.  The  eye- 
lids hang  and  cannot  be  closed  completely.  The  shoulder-girdle  atrophy 
then  advances,  the  waist  is  small,  wasp-like,  the  chest  flattened;  finally 
the  dystrophic  process  becomes  universal. 


Fig.  170. — Pseudohypertrophic  myopathy.     (G.  Hammond.) 

4.  Amyotonia  Congenita — ^Myotonia  Congenita — Oppenheim^  (Wil- 
son).— It  is  not  certain  whether  this  disorder  should  be  included 
among  the  dystrophies  or  not.  Pathologically  it  seems  identical, 
clinically  it  is  quite  at  variance.  Spiller  made  the  first  autopsy.  The 
number  of  cases  known  (about  60 — 1911)  prevents  a  definite  answer 
at  the  present  time.^  Rothmann  is  inclined  to  ally  it  with  the  Werdnig- 
Hoifmann  spinal  nuclear  atrophies  as  a  congenital  variety. 

Symptoms. — ^The  disorder  is  usually  congenital,  hypotonia  is  char- 
acteristic, with  loss  of  tendon  reflexes.    Active  motion  is  impaired  by 


»  Monats.  f.  Neurologie  u.  Psychiatrie,  8,  1900,  p.  232. 

2  Latest  literature:  ColHer  and  Holmes,  Brain,  1909;  Batten,  loc.  cit.;  Cassirer,  Hand- 
buch  d.  Neur.,  1911;  Griffith,  Arch.  Kindhk.,  1910;  Griffith  and  Spiller,  Am.  Jour.  Med. 
Sci.,  August,  1911. 


MUSCULAR  DYSTROPHIES 


335 


reason  of  weakness,  but  the  limbs  are  not  paralyzed.  Usually  the 
lower  limbs  are  involved,  in  half  of  the  cases  the  upper,  and  in  a  few, 
those  of  the  trunk  and  neck.  These  little  patients  kaleidoscope  as  it 
were.  The  facial  muscles  are  usually  spared.  The  intercostals  are 
but  little  affected. 

Electrical  reactions  are  normal,  or  show  quantitative  reduction. 
The  knee  and  Achilles  jerks  are  usually  absent;  those  of  the  upper 
extremity  less  constantly  gone.  Atrophy  is  not  definite,  nor  is  pseudo- 
hypertrophy present.  Mechanical  irritability  and  fibrillary  contractions 
are  absent. 


Fig.  171. — Landouzy-Dejerine 
myopathy. 


Fig.  172. — Landouzy-Dejerine 
myopathy. 


Contractures  are  not  uncommon.  The  sphincters  are  intact.  Sen- 
sibility is  intact,  also  the  special  senses,  and  the  children  are  usually 
bright  mentally.  The  general  condition  is  good,  and  vasomotor 
disturbances  are  absent. 

The  disorder  has  some  tendency  to  improve,  although  Batten 
claims  that  not  one  has  got  well.  Some  of  the  patients  learn  to 
stand,  but  rarely  unaided. 

Intercurrent  disorders,  particularly  respiratory,  cause  death  in  the 
majority. 

5.  Distal  Type. — Gowers-Spiller,^  and  Spiller^  first  definitely  sepa- 
rated this  myopathy  from  its  apparently  related  Charcot-Marie-Tooth 


1  Brit.  Med.  Jour.,  1902. 


2  Jour.  Nerv.  and  Ment.  Dis.,  1906. 


336 


LESIONS  OF  THE  SPINAL  CORD 


atrophy.    It  varies  little  from  this  latter  save  in  the  absence  of  sensory 
disturbances. 

6.  Myotonia  Atrophica.^ — A  combination  of  muscular  atrophy  with 
slow  relaxation  of  the  muscles — possibly  more  closely  related  to 
Thomsen's  disease  than  to  the  dystrophies  (Pelz-atypical  Thomsen's 


Fig.   173. — Myatonia  atrophica.      Note  dropped  feet,  atrophy  of  hand   muscles,  and 

facies.     (Abrahamson.) 

disease).  The  distribution  of  the  atrophies  is  peculiar,  corresponding 
to  none  of  the  well-known  types  of  myopathies.  There  is  usually 
weakness  of  the  facial  muscles,  also  masseters  and  temporals  (50  per 
cent.),  atrophy  of  the  sternomastoids,  the  vasti,  and  dorsiflexors  of 


1  Fiirnrohr,  Deutsche  Zeitschrift  f.  Nervenheilkunde,  1907,  33. 


AMYOTROPHIC  LATERAL  SCLEROSIS  337 

the  feet.  Considerable  variation  in  the  distribution  is  known. ^  The 
intrinsic  muscles  of  the  hand  are  usually  preserved.  Batten  and  Gibb 
have  collected  all  of  the  cases  to  1909. 

AMYOTROPHIC   LATERAL    SCLEROSIS. 

History. — The  position  of  the  progressive  muscular  atrophies,  with 
their  many  clinical  variations,  and  the  anomalous  pathological  find- 
ings is  still  in  need  of  more  detailed  analysis.  Charcot,  in  1865,  sepa- 
rated from  this  mass  a  special  group  which  combined  the  features 
of  a  poliomyelitis  and  a  lateral  sclerosis.  Atrophy  with  spasticity 
was  the  determining  feature,  and  in  1872-1874  after  previous  studies 
with  Joffroy  and  Gombault  he  gave  it  definite  form.  Dejerine  later 
(1883)  showed  that  the  glosso-labio-laryngeal  palsies  of  Duchenne 
were  a  constituent  part  of  the  disease,  and  the  work  of  Kojewnikoff, 
Marie,  Roth,  Florand,  Holmes,  Spiller,  Raymond,  Cestan  and  others 
has  served  to  establish  this  disorder  upon  a  sound  pathological  and 
clinical  basis. 

It  is  a  disease  of  the  entire  motor  neuron,  although  Holmes  has 
shown  that  this  is  not  universal.  Schultze  has  therefore  suggested 
the  term  motor  tabes  in  contrast  with  sensory  tabes  of  the  classical 

type. 

Etiology. — ^This  is  a  comparatively  rare  disease.  Little  is  known 
concerning  its  causes.  It  has  been  thought  of  as  a  primary  atrophy 
of  the  motor  system — an  abiatrophy,  but  this  is  only  pushing  the 
explanation  back  a  step  further.  It  is  most  prevalent  in  early  adult 
life,  thirty  to  forty,  but  it  is  also  known  to  occur  in  children  (Erb-Seelig- 
miiller),  and  Soques,  Roussy,  Raymond,  Probst  and  others  have 
described  cases  occurring  in  the  fifth  decade.  Women  seem  to  have 
the  disease  somewhat  oftener  than  men,  but  the  differences  are  not 
striking.  The  statistics  of  the  Vanderbilt  Clinic  for  ten  years  show  the 
reverse  conditions — 1 1  men  and  6  women. 

Arteriosclerosis,  trauma,  infection,  intoxication,  syphilis  have  each 
been  shown  to  play  some  role  in  the  causation  in  some  patients. 

Pathology. — While  the  disease  is  one  in  which  the  entire  motor 
system  is  predominantly  affected,  this  is  not  exclusively  so,  as  Holmes 
has  definitely  shown.  The  spinal  motor  neurons  are  chiefly  affected, 
both  centrally  and  peripherally.  The  anterior  horn  cells  are  degen- 
erated, and  the  motor  nerve  fibers  as  well. 

The  corticospinal  tracts  are  also  degenerated.  This  degeneration 
can  be  followed  to  the  Betz  cells  of  the  Rolandic  cortex  in  practically 
all  of  the  cases  of  any  duration,  but  in  some  patients  the  degeneration 
in  the  pj^amidal  tracts  has  not  been  traced  beyond  the  pons. 

Degeneration  in  the  bulbar  motor  nuclei  is  the  rule,  and  in  the  cortico- 
bulbar  tracts,  as  was  demonstrated  by  J.  Dejerine  in  1883.    The  third, 

1  Batten  and  Gibb,  Brain,  1909. 
22 


338 


LESIONS  OF   THE  SPINAL  CORD 


fourth,  and  sixth  nerve  nuclei  usually  escape,  though  a  few  recorded 
degenerations  here  are  known  (Hoche,  Pal,  etc.)- 

Degenerations  can  also  be  traced  in  the  corpus  callosum.  Following 
the  degeneration  there  is  some  replacement  sclerosis.  This  widespread 
degeneration  in  the  motor  system  may  be  accompanied  by  other 
degenerations,  however,  although  such  degenerations  seem  to  stand  out 
less  prominently  in  the  clinical  picture.  Thus,  degenerations  in  the 
lateral  columns,  Clarke's  columns,  posterior  longitudinal  bundles, 
spinocerebellar  tracts,  and  ventrolateral  ground  bundles  are  recorded. 

Symptoms. — The  disorder  may  begin  in  almost  any  motor  nerve 
region:  bulbar,  cervical,  dorsal  or  lumbar;  more  than  one  area  may 
be  initially  affected  simultaneously,  but  the  usual  beginning  lesions  are 
predominantly  in  the  muscle  groups  of  the  cervical  cord.  The  order 
of  involvement  is  predominantly  radicular. 


Fig.  174. — ^Amyotrophic  lateral  sclerosis  showing  atrophy  of  the  arms. 


The  usual  symptoms  are  muscular  weakness,  with  wasting  of  the 
muscles,  usually  distally,  with  increased  reflex  excitability.  The 
patients  first  note  that  the  hands  get  tired,  they  are  clumsy  in  dress- 
ing and  undressing.  Buttoning  and  unbuttoning,  sewing,  writing, 
and  the  carrying  out  of  the  more  delicate  finger  manipulations  become 
increasingly  difficult,  first  from  fatigue,  then  from  stiffness  and  motor 
palsy.  Atrophy  of  the  thenar  and  hypothenar  eminences  occurs,  then 
progresses  up  the  muscles  of  the  forearm,  arm,  and  shoulder-girdle. 
Weakness  and  stiffness  then  make  themselves  evident  in  the  lower 
extremities,  and  a  similar  progression  takes  place.  Then  after  months 
or  a  year  or  so  the  muscles  of  the  lips,  of  the  tongue,  and  swallowing 
and  phonation  gradually  become  implicated.    The  lips  become  thinned. 


AMYOTROPHIC  LATERAL  SCLEROSIS  339 

the  tongue  atrophies,  speech  fatigue  is  followed  by  inability  to  speak; 
swallowing  occurs  with  regurgitation,  cardiac  irregularities  develop, 
and  the  patients  die  of  aspiration  pneumonia,  weakness,  or  other 
intercurrent  affections. 

With  the  atrophies  there  develops  a  gradually  increasing  spasticity. 
The  tendon  reflexes  are  increased,  there  is  increased  jaw-jerk,  or 
mandibular  clonus;  the  biceps  and  triceps,  and  scapular  reflexes  are 
increased,  patellar  clonus,  increased  knee-jerks,  increased  Achilles, 
ankle  clonus,  Babinski  and  Oppenheim  reflexes  are  manifest. 

The  muscles  themselves  show  increased  myotatic  irritability, 
fibrillary  contractions  are  common,  or  the  atrophies  may  be  so  marked 
that  flaccid  conditions  develop.  The  electrical  reaction  of  degeneration 
occurs. 

Sensory  anomalies  are  extremely  rare.  Pain  is  probably  present 
at  some  periods  in  many  cases;  it  is  that  of  fatigue  or  spasm,  however. 
The  bladder  and  rectal  functions  are  not  usually  involved,  and  the 
majority  of  the  patients  show  only  the  involvement  of  the  motor 
neurones. 

Mental  symptoms  are  not  prominent;  increased  emotionalism  is 
usually  present,  and  as  the  patients  are  often  much  depressed,  the 
involuntary  crying  which  is  very  frequent  is  augmented  thereby. 

As  has  been  noted,  the  onset  may  be  in  any  part  of  the  motor 
system — thus  the  disorder  may  progress  for  some  time,  even  to  death, 
as  a  bulbar  type  of  palsy,  with  increased  reflexes  in  the  motor  cranial 
nerves;  death  occurring  in  some  instances  before  there  are  any  spinal 
symptoms.  Again  the  distribution  may  be  hemiplegic,  and  then 
triplegic,  and  finally  quadriplegic  and  bulbar. 

Again  the  disorder  may  bear  the  stamp  of  a  lower  extremity  para- 
plegia, or  an  upper  limb  diplegia;  again,  whereas  the  distal  muscles 
are  usually  initially  involved,  patients  show  proximal  atrophies  in 
either  upper  or  lower  extremities;  and  the  more  cases  reported  the 
greater  are  the  possibilities  encountered  in  the  way  of  anomalous 
localizations. 

But  throughout,  after  a  certain  lapse  of  time,  the  combination  of 
weakness,  atrophy,  increased  reflex  activity,  without  sensory  changes, 
stamps  the  process  as  essentially  a  motor  degeneration,  and  whereas 
one  even  finds  the  spastic  phenomena  antedate  the  atrophic  changes, 
yet  in  the  end  the  two  level  up,  unless  the  course  has  been  unusually 
active,  and  death  results  early  from  the  disease  or  from  inter- 
current disorder.  Here  the  pathological  picture  may  seem  to  contra- 
dict the  clinical  findings,  and  the  interpretation  remains  that  the  patient 
did  not  live  long  enough  to  show  the  classical  syndrome. 

On  account  of  this  polymorphism,  certain  authors  have  erected 
types  which  may  be  useful  clinically,  but  which  are  rarely  borne 
out,  save  for  certain  periods,  during  the  development  of  the  dis- 
ease. Thus  Raj-mond  and  Cestan  (R.  N.,  1905)  make  (1)  an 
ordinary  spinal  type   with  the   classical   main  en  griffe  of   Charcot, 


340  LESIONS  OF   THE  SPINAL  CORD 

increased  reflexes,  particularly  in  the  upper  extremities,  less  marked 
in  the  lower,  with  occasional  failure  of  the  Babinski  reflex;  (2)  labio- 
glosso-laryngeal  type — apparently  more  frequent  in  women — ^which 
begins  in  the  muscles  of  the  lips,  tongue,  pharynx,  or  larynx,  occasion- 
ally with  facial  palsies,  inability  to  close  the  eyes,  etc.,  with  fibrillary 
twitchings,  lively  mas seter- jerk.  Some  of  these,  but  not  all,  run  a 
rapidly  fatal  course  from  four  to  nine  months,  and  die  before  the  lower 
neurons  show  their  characteristic  changes;  (3)  an  amyotrophic  type 
with  pronounced  atrophies  which  overshadow  or  mask  the  spasticities 
and  the  increased  reflexes.  These  patients  resemble  chronic  polio- 
myelitis, often  for  years,  and  constitute  a  most  difficult  group  to  differ- 
entiate. (4)  A  spastic  type,  in  which  the  reverse  condition  exists, 
and  increased  reflexes  and  spasms,  contractures,  etc.,  antedate  the 
atrophies.     These  resemble  multiple  sclerosis,  lateral  sclerosis,  etc. 

Finally  it  should  be  recognized  that  certain  patients  represent 
transition  forms;  they  are  not  pure  types,  and  the  apparent  clinical 
picture  of  amyotrophic  lateral  sclerosis  is  due  to  other  than  the  recog- 
nized pathological  foundations  of  this  disorder. 

Course  and  Duration. — The  average  time  of  duration  of  some  hundred 
or  more  cases  analyzed  is  about  two  years;  but  this  by  no  means  tells 
anything  about  any  individual  case.  Death  in  four  months  after 
the  onset  has  been  the  result  in  a  score  or  more  of  cases,  some  have 
persisted  ten  years,  and  a  few  questionably  diagnosed  cases  even  much 
longer.  The  rule  is  toward  steady  progression;  there  are  few  leaps 
and  regressions,  as  is  seen  in  multiple  sclerosis,  and  so  far  as  is  known 
the  disease  is  a  fatal  one.  A  few  stationary  cases  are  reported.  Bulbar 
palsies,  when  not  among  the  initial  symptoms,  usually  develop  before 
the  second  year,  and  are  often  the  cause  of  death  by  choking,  or 
dyspnea.  Cardiac  irregularities,  pneumonia,  exhaustion,  secondary 
infections,  these  are  the  usual  modes  of  death.     Suicide  is  unusual. 

Diagnosis. — The  chief  disorders  needing  separation  are  multiple 
sclerosis  and  progressive  spinal  muscular  atrophy  or  chronic  polio- 
myelitis. The  former  rarely  shows  atrophies,  and  the  eye-grounds, 
ocular  muscle  signs,  and  characteristic  tremors  should  separate  this; 
the  latter  disorder  when  it  shows  increased  reflexes,  as  is  occasionally 
the  case,  is  separated  with  great  difficulty.  Lumbar  puncture  does 
not  throw  any  light  on  the  diagnosis. 

Treatm.ent. — No  specific  is  known.  Overfeeding,  light  massage, 
much  rest,  caffein,  strychnin  are  the  only  methods  at  present  of  service. 
Certain  anomalous  thyroid  insufficiencies  have  been  associated  with 
progressive  muscular  wasting,  and  indicate  a  field  for  experimental 
therapeutics.  Parathyroid  myatonias,  myasthenias,  family  periodic 
paralyses,  etc.,  offer  a  suggestion  in  this  line  of  a  perverted  parathyroid 
activity  that  may  be  seized  upon  for  the  study  of  the  mineral 
metabolism,  especially  of  calcium.  Personal  cases  treated  along  lines 
suggested  by  the  mineral  metabolism  of  the  body  offer  a  glimmer  of 
hope  in  a  hitherto  hopeless  disease.  Syphilitic  cases  need  specific 
therapy. 


FRACTURE  AND  DISLOCATION  SYNDROMES 


HI 


FRACTURE  AND  DISLOCATION  SYNDROMES. 

Spinal  cord  injuries  result  from  bullets,  penetrating  instruments, 
or  from  blows  or  falls. 

Bullets  and  cutting  instruments  cause  hemorrhage,  with,  as  a  rule, 
partial  or  complete  severance  of  the  cord,  with  septic  infections  of  the 
cord  and  meninges.  Blows  and  hard  falls  occasion  fractures  or  disloca- 
tions, with  crushing  of  the  cord  to  a  greater  or  lesser  degree.  Mild 
injuries  may  result  merely  in  the  bruising  of  the  cord,  or  minute 


Fig.  175. — Total  cross-section  of  spinal  cord  at  the  level  of  d  showing  the  results 
upon  the  long  fiber  tracts.  Middle  section  indicates  level  of  injury.  Cross-hatching 
above  and  below  indicate  the  degenerations  or  loss  of  function.     (Veraguth.) 


hemorrhages  within  the  cord  or  of  the  pial  or  dural  spaces  only  may 
be  produced,  sometimes  even  from  excessive  exertion,  long  marching, 
severe  athletic  exercises,  sudden  spinal  torsions,  etc.  Dislocation 
of  a  vertebra  practically  always  causes  a  crushing  of  the  cord;  the 
so-called  dislocations  without  spinal  cord  injury  are  more  apt  to  be 
wrenches  of  the  vertebrae  or  very  limited  dislocations.  Fractures 
may  result  with  but  few  spinal  symptoms;  crushing  of  the  cord  causes 
more  or  less  disintegration,  usually  accompanied  by  severe  hemorrhages 
within  or  without  the  cord  substances  (hematomyelia,  dural  hemor- 
rhage).    Hematomyelia  usually  extend  up  and  down  the  cord  from 


342 


LESIONS  OF  THE  SPINAL  CORD 


the  point  of  destruction,  sometimes  involving  several  segments  of  the 
cord. 

Symptoms. — Fractures  or  dislocations  of  the  spinal  vertebrse  usually 
cause  local  deformity,  much  pain  and  muscular  rigidity,  particularly 
on  motion.  X-ray  examination  reveals  the  nature  and  extent  of  the 
bony  lesion.  The  motor  and  sensory  phenomena  at  the  level  and  below 
the  site  of  the  lesion  determine  the  location,  extent  and  character  of 


Figs.  176  and  177. — -Showing  superficial  sensibility  disturbances  in  complete  transverse 
lesions  of  the  cord  at  the  levels  of  Ct,  Cs,  -De,  and  Ls,  respectively.     (Veraguth.) 


the  injury  to  the  spinal  cord.  The  symptoms  usually  develop 
immediately  following  the  injury,  become  slightly  progressive,  if 
hemorrhage  only,  and  then  slowly  recede  until  after  a  variable  length 
of  time,  usually  from  two  or  three  months  the  residual  symptoms 
indicate  the  permanent  impairment  of  function. 

The  chief  syndromes,  which  develop  from  injury  to  the  spinal  cord 
at  different  levels,  are  here  described  by  means  of  charts.  A  careful 
sensory  examination  is  indispensable  and  should  follow  the  directions 
already  laid  down. 


FRACTURE  AND  DISLOCATION  SYNDROMES 


343 


It  is  important  to  recall  that  the  spinal  cord  segments  and  the  verte- 
bral segments,  while  nearly  corresponding  during  infancy,  do  not  in 


General  Func- 
SiTE  OP  Lesion.  tion. 


Initial  Syndrome. 


Residual  Syndrome. 


Motility. 


Symmetrical  flaccid  palsy  of 
the  lower  extremities 


Symmetrical  spastic  paralysis 
of  lower  extremities  to  level 
of  L.  4. 


Lumbar  i 
(L.  J.) 


Symmetrical  flaccid  palsy  of 
the  abdominal  muscles  and 
lower  extremities. 


Symmetrical  spastic  palsy  of 
the  abdominal  muscles  and 
lower  extremities. 


Symmetrical  palsy  of  preced- 
ing plus  flaccid  palsy  of 
intercostals. 


Symmetrical   spastic   palsy   of 
preceding    plus   intercostals. 


Sensory  i 


Jorsal  Segmejitg 
(D.  6.) 


Preceding  signs  plus  flaccid 
palsy  of  upper  limbs  and 
disturbances  of  breathing. 

Symmetrical  loss  of  superfi- 
cial and  deep  sensibility  of 
the  lower  extremities.  Re- 
tention of  spinal  sensory 
functions  below  lesion. 

Preceding  plus  loss  of  super- 
ficial and  deep  sensibilities 
between  D.  6  and  L.  3. 


Preceding  plus  loss  of  super- 
ficial and  deep  sensibilities 
between  D.  1  and  D.  6. 


When  not  resulting  in  death 
total  bilateral  spastic  par- 
aly.'iis. 


Preceding  plus  loss  of  super- 
ficial and  deep  sensibility 
between  C.  4  and  D.  1. 


Cervlco  Dors^ilX 
CCS.  £»i.) 


IS(i,i/iclri  MotAr  (  Total  loss  of  patellar,  Achilles, 
plantar  reflexes.  Cremas- 
teric preserved. 


Preceding  plus  loss  of  abdom- 
inal reflexes. 


Patellar  reflex  regained.  Achilles 
increased.  Clonus  and  Bab- 
inski  and  contralateral  Bab- 
inski  obtained. 


Preceding     plus     spontaneous 
abdominal  movements. 


Preceding     with    oculopupil- 
lary  disturbances. 


Preceding  with   oculopupillary 
disturbances. 


Cervical 
(C.4.) 


Trophic 
DisturBances 


Preceding  without  oculo- 
pupillary signs  but  with 
loss  of  reflexes  of  upper 
extremities. 

Total  bladder  and  intestinal 
palsy. 


Symmetrical     dilatation     of 
vessels  of  lower  extremity. 


Preceding  with  vascular  dila- 
tation in  upper  extremities. 


Preceding  without  oculomotor 
disturbance  and  increased 
reflexes  of  upper  extremities. 


Total  bladder  and  intestinal 
palsy  with  later  hyperirri- 
tability;  also  of  genital 
reflexes  without  priapism. 

Objective  symmetrical  chilli- 
ness and  cyanosis  of  the  legs. 
Later  sacral  decubitus.  Skin 
and    nail    disturbances. 

Preceding  with  objective  sym- 
metrical cold  and  cyanosis 
of  the  arms.  Shoulder-girdle 
decubitus  possible. 


Fig.  178. — Localization  of  symptoms  at  different  levels  of  the  cord.     (Veraguth.) 


344  LESIONS  OF   THE  SPINAL  CORD 

the  adult.  It  is  rare  that  absolutely  symmetrical  involvements  result 
from  spinal  injury.     Usually  one  side  is  involved  more  than  the  other. 

A  total  destruction  of  the  spinal  cord  will  result  in  initial  and  in 
residual  symptoms.  This  may  result  from  accident,  bullet  wound, 
diving,  caisson  disease,  myelitis,  tumor — Pott's  disease  chiefly.  The 
course,  progression  and  mode  of  treatment  will  depend  entirely  upon 
the  causative  lesion.  The  chief  symptoms  of  acute  cross-lesion  are 
expressed  in  the  table  on  page  343  and  diagrams  according  to  the  site 
of  the  lesion. 

Lesions  of  the  upper  cervical  segments  usually  causes  instant  death 
from  respiratory  paralysis.  Injury  lower  down  is  very  frequent, 
occurring  in  workmen  from  objects  falling  upon  the  bent  neck  or  in 
reckless  diving.  The  symptoms  are  charted.  Many  of  these  patients 
live  for  some  time,  even  years,  dying  usually  from  bladder  and  kidney 
complications.  Dorsal  lesions  are  comparatively  rare,  and  often 
show  the  Brown-Sequard  syndrome,  which  latter  may,  however, 
develop  from  lesions  at  all  levels  above  the  conus. 

Lower  dorsal  and  upper  lumbar  lesions  are  the  most  frequent  of  all. 
Their  symptoms  are  indicated  in  the  chart. 

Injury  to  the  lower  lumbar  vertebrae  causes  cauda  equina  lesions,  as 
the  cord  proper  terminates  at  about  the  first  lumbar. 

Intramedullary  lesions — hematomyelia — have  a  symptomatology  all 
their  own. 

Careful  sensory  testing  shows  that  in  an  intramedullary  lesion  there 
is  a  complete  separation  of  the  impulses  underlying  the  appreciation  of 
posture,  the  discrimination  of  two  points,  and  their  correlated  faculties 
from  those  of  other  sensory  groups. 

All  painful  and  thermal  impulses  coming  from  the  periphery,  undergo 
regrouping  after  entering  the  spinal  cord,  and,  whether  they  arise 
in  the  skin  or  in  deeper  structures,  become  arranged  according  to 
functional  similarity.  Then,  after  a  longer  or  shorter  course,  they 
pass  away  to  the  opposite  side  of  the  spinal  cord.     (See  Plate  XL) 

This  process  of  filtration  leaves  all  the  impulses  associated  with 
postural  and  spacial  recognition  to  continue  their  course  unaltered 
in  the  posterior  columns;  they  are  the  survivors  of  peripheral  groups 
broken  up  by  the  passing  away  of  certain  components  into  secondary 
afferent  systems.  At  any  point  in  the  spinal  cord,  these  columns  trans- 
mit not  only  impulses  from  the  periphery  which  are  on  their  way,  after 
a  shorter  or  longer  passage,  to  regrouping  and  transformation,  but  at 
the  same  time  they  form  the  path  for  impulses,  arising  both  in  the 
cutaneous  and  deep  afferent  systems,  which  undergo  no  regrouping 
until  they  reach  the  nuclei  of  the  medulla  oblongata. 

Thus,  a  lesion  confined  to  one-half  of  the  spinal  cord,  even  at  its 
highest  segment,  may  interfere  with  the  passage  of  sensory  impulses, 
some  of  which  are  travelling  in  secondary  paths,  whilst  others  are  still 
within  the  primary  level  of  the  nervous  system.  All  impulses  concerned 
with  painful  and  thermal  sensations  from  distant  parts,  disturbed  by 


FRACTURE  AND  DISLOCATION  SYNDROMES  345 

such  a  lesion,  will  be  travelling  in  secondary  paths  and  will  have  come 
from  the  opposite  half  of  the  body;  for,  after  regrouping,  they  have 
passed  across  the  spinal  cord.  But  those  impulses  underlying  the 
appreciation  of  posture,  the  compass  test,  size,  shape,  form,  weight, 
consistence,  vibration,  will  be  affected  on  the  same  half  of  the  body 
as  the  lesion.  They  still  remain  in  paths  of  the  primary  level  and 
have  undergone  no  regrouping.     (See  Plates  X  and  XL) 

In  such  a  case  the  parts  on  the  side  opposed  to  the  lesion  may  be 
insensitive  to  pain,  heat  and  cold;  but  all  the  postural  and  spacial 
aspects  of  sensation  will  be  perfectly  maintained.  Yet,  all  power  of 
recognizing  position,  of  estimating  size,  shape,  form  and  weight,  or  of 
discriminating  the  two  compass  points,  will  be  lost  in  the  limbs  which 
lie  on  the  side  of  the  lesion,  although  tactile  sensibility  and  localization 
of  the  spot  stimulated  may  be  perfectly  preserved. 

This  remarkable  arrangement  enables  one  to  analyze,  as  Head  has 
pointed  out,  the  nature  of  the  peripheral  impulses  upon  which  depend 
the  power  of  postural  and  spacial  recognition.  Obviously,  even  at 
the  periphery,  they  must  be  independent  of  touch  and  pressure.  The 
power  to  distinguish  two  points  applied  simultaneously  and  to  recog- 
nize such  size  and  shape,  requires  as  a  preliminary  the  existence  of 
sensations  of  touch;  but  the  patient  may  be  deprived  of  all  such  powers 
of  spacial  recognition  without  any  discoverable  loss  of  tactile  sensibility. 
In  the  same  way  our  power  to  appreciate  the  position  of  a  limb,  or  to 
estimate  the  weight  of  an  object,  is  based  upon  impulses  which,  even 
at  the  periphery,  exist  apart  from  those  of  touch  and  pressure,  called 
into  simultaneous  being  by  the  same  external  stimulus. 

This  long  delay  of  the  postural  and  spacial  elements  in  reaching 
secondary  paths  enables  them  to  give  off  afferent  impulses  into  the 
spinal  and  cerebellar  coordinating  mechanisms,  which  lie  in  the  same 
half  of  the  spinal  cord.  The  impulses  which  pass  away  in  this  direction 
are  never  destined  to  enter  consciousness  directly.  They  influence 
coordination,  unconscious  posture  and  muscular  tone,  and,  although 
arising  from  the  same  afferent  end-organs,  they  never  become  the  basis 
of  a  sensation. 

Finally,  the  last  survivors  of  these  impulses  from  the  periphery 
become  regrouped  in  the  nuclei  of  the  posterior  columns  and  cross  to 
the  opposite  half  of  the  medulla  oblongata  in  paths  of  the  secondary 
level.  So  they  pass  to  the  optic  thalamus  and  thence  to  the  cortex,  to 
underlie  those  sensations  upon  which  are  based  the  recognition  of 
posture  and  spacial  discrimination. 

Diagnosis. — Accurate  testing  of  the  motor  and  sensory  functions 
leaves  no  doubt  as  to  the  presence  of  a  spinal  cord  injury  of  a  major 
grade.  The  results  of  minute  lesions  may  escape  recognition.  Total 
severance  of  the  cord  is  unusual,  most,  even  severe  injuries,  leave  some 
pathways  undivided,  which,  after  the  immediate  effects  of  the  injury 
have  subsided,  give  some  sensory  response.  Total,  complete  severance 
causes  absolute  anesthesia  to  all  forms  of  stimuli,  flaccid  paraplegia, 


346 


LESIONS  OF   THE  SPINAL  CORD 


with  loss  of  all  reflexes  and  all  visceral  reactions  below  the  site  of  the 
lesion. 

Prognosis. — This  is  bad  in  practically  all  spinal  cord  injuries  save 
small  hematomyelia  or  limited  cauda  equina  lesions.  High  lesions 
are  almost  immediately  fatal.  Lower  lying  ones — fourth  cervical  and 
down — vary  in  their  immediate  and  remote  results,  according  to  the 
site  of  the  lesion  and  its  extent. 


Fig.  179. — Lesion  of  the  cord  producing  a  Brown- 
Sequard  syndrome.     (Veraguth.) 


Fig.  180. — Brown-Sequard  syn- 
drome. Black  represents  super- 
ficial anesthesia  in  joint  anesthesia; 
—  passing  hyperesthesia  and  par- 
alysis.    (Veraguth.) 


The  question  of  regeneration  of  spinal  neurons  has  not  as  yet  been 
entirely  settled  experimentally,  but  the  preponderance  of  practical 
evidence  is  against  it.  Pathways  once  destroyed  remain  so.  Just 
at  what  period  it  may  be  said  that  the  residual  symptoms  will  be 
permanent  is  largely  a  matter  of  the  extent  of  the  lesion.  As  a  rule 
the  findings  at  three  months  are  apt  to  be  those  of  permanence,  still 
occasionally  marked  improvement,  particularly  in  bladder  and  rectal 
functions,  may  take  place  after  a  year  or  more  of  total  loss.    Many 


FRACTURE  AND  DISLOCATION  SYNDROMES 


347 


spinal  lesions,  especially  those  dependent  chiefly  on  hemorrhage,  will 
show  widespread  symptoms  early,  and  later  these  will  be  reduced  to  a 
minimum.  The  chief  bugbear  as  to  life  is  the  bladder.  Great  care 
should  be  taken  of  it  in  the  early  stages,  and  every  available  measure 
taken  to  keep  it  sterile. 


Site  of 
Lesion. 


Initial  Syndrome. 

On  the  side  of  the  lesion.  Flac- 
cid palsy  of  muscles  whose 
neurons  lie  caudad  to  lesion. 

Normal  on  opposite  side. 

On  the  side  of  the  lesion.  Disturb- 
ance of  deep  sensibility,  espe- 
cially joint  hypesthesia,  caudad 
to  affected  metameres.  Small 
zone  of  superficial  anesthesia 
above  the  palsied  motor  zone. 

Hypesthesia  for  touch  caudad 
to  affected  metameres. 

On  the  crossed  side,  and  partly 
on  the  side  of  lesion,  because 
of  limitations  in  crossing  space 
of  fibers  below  lesion,  superfi- 
cial hypesthesia,  especially 
thermohypesthesia  and  hypal- 
gesia. 
*^  As  above. 

On  the  side  of  the  lesion.  Loss 
of  tendon  and  skin  reflexes  of 
the  lower  extremity.  Babin- 
ski  phenomenon. 

On  opposite  side.  Loss  of  skin 
reflexes  of  the  lower  extremity. 

Preceding  and  loss  of  abdominal 
reflex. 

Preceding  and  loss  of  tendon 
reflexes  of  the  upper  extremity 
of  the  side  of  the  lesion. 

On  the  side  of  the  lesion  the 
skin  of  the  caudad  metameres 
red  and  hot. 


On  the  crossed  side  normal. 
Preceding  plus  oculopupillary 
signs    on    affected    side. 


Fig.  181. — Brown-Sequard  lesions. 


Residual  Stndhome. 

On  side  of  lesion,  spas- 
tic palsy  of  muscles 
whose  neurons  lie 
caudad    to    lesion. 

Normal  on  side  oppo- 
site to  lesion. 


Hyperesthesia 
pears  shortly. 


disap- 


On  the  side  of  the 
lesion.  Increase  of  ten- 
don and  skin  reflexes. 

Babinski.  Contralat- 
eral Babinski. 


Preceding  and  increased 
abdominal  reflexes  on 
side  of  lesion. 

Preceding  plus'  hand 
clonus,  rotation  clo- 
nus, etc.,  on  affected 
side. 

On  side  of  lesion  the 
skin  of  the  caudad 
metameres  either 

normal  or  cyanotic 
or    objectively     cool. 

On  crossed  side  nor- 
mal. Preceding  with 
oculopupillary  signs 
on  the  side  of  lesion. 


Operations,  after  spinal  injury,  are  often  futile,  yet  with  care  rarely 
do  any  harm  and,  not  infrequently,  if  the  cord  itself  is  only  being 
pressed  upon  by  hemorrhage,  may  be  distinctly  advantageous.  Evi- 
dent surgical  indications  (fracture,  dislocation)  should  be  met.  The 
patient  is  rarely  benefited  but  is  entitled  to  the  opportunity. 


348  LESIONS  OF   THE  SPINAL  CORD 

Therapy. — Surgical  treatment  is  indicated  in  most  spinal  injuries 
even  though  the  prognosis  is  not  at  all  reassuring.  The  persistence  of 
some  form  of  sensibility  (all  types  should  be  tested)  is  an  indication 
that  the  cord  is  not  completely  severed.  Operation  during  shock  is  not 
advisable,  yet  too  long  a  delay  is  unwarranted,  even  if  all  sensibility 
seems  abolished  below  the  lesion.  The  functional  loss  practically 
always  exceeds  the  anatomical  defect.  X-ray  examination  should  be 
made  immediately  for  diagnosis,  and  if  there  is  evidence  of  compression, 
operation  is  advisable  as  early  as  is  compatible  with  the  patient's 
condition.  In  the  absence  of  evidence  of  compression,  earlier  surgeons 
advised  against  operation  as  dangerous  and  futile.  Since  in  the  hands 
of  competent  surgeons  most  of  the  dangers  of  spinal  cord  injury 
have  disappeared,  there  is  a  growing  tendency  to  operate  more  freely 
and  at  times,  fortunately,  even  though  more  often  without  any  gain. 

The  general  treatment  of  the  patient  is  of  great  importance.  It 
should  be  directed  toward  giving  a  maximum  of  relief  from  distress; 
avoidance  of  bladder  infection,  preventing  bed-sores,  and  careful 
bowel  attention.  Antispasmodics  and  analgesics  may  have  to  be  used 
for  a  long  time,  but  morphin  and  its  derivatives  are  to  be  avoided  if 
possible,  save  in  those  agonizing  cases  where  it  gives  the  only  relief 
from  torture.     Electricity  is  largely  a  placebo. 

Half-sided  lesions  produce  the  typical  Brown-Sequard  syndromes, 
which  vary  according  to  the  segments,  as  seen  in  the  accompanying 
scheme  (Fig.  181.) 

COMPRESSION  OF  THE  CORD. 

Compression  of  the  cord,  as  a  slow  chronic  process  results  from  (1) 
bon}^  hj^pertrophies,  (2)  tuberculosis,  (3)  tumors,  (4)  syphilis,  (5) 
aneurisms,  (6)  meningeal  disease. 

Bony  Hypertrophies  (Osteitis,  Osteitis  Deformans,  Osteo-arthritis, 
Spondylosis  Rhizomelique) . — Under  these  various  names  one  finds 
patients  who  present  signs  of  more  or  less  spinal  cord  compression, 
either  with  or  without  stiff  backs  or  deformities. 

They  show  gradually  increasing  weakness,  going  on  to  paresis, 
or  complete  paraplegia  with  spasticity  and  increased  reflexes.  Fre- 
quently there  is  severe  pain,  and  when  the  bony  disease  impinges  upon 
the  intervertebral  foramina  neuritic  pains  and  symptoms  of  peripheral 
nerve  palsy  develop.  (See  Plexus  Palsies.)  X-ray  examinations  reveal 
the  nature  of  the  bony  changes  and  the  location  of  the  pressure. 

Tuberculosis  (Caries). — This  is  a  most  widespread  cause  of  spinal 
cord  compression.  The  tuberculous  focus  usually  begins  within  the 
body  of  the  vertebrae;  breaking  down  and  destruction  of  the  vertebrae 
take  place,  with  displacement  and  projection  of  the  vertebrae  either 
forward,  backward  or  laterally,  causing  the  various  deformities  of 
Pott's  disease.  The  tuberculous  process  usually  spreads  to  the  spinal 
meninges   (tuberculous  pachymeningitis).     Thus  if  the  compression 


COMPRESSION  OF   THE  CORD  349 

arises  it  may  be  from  both  processes.  Even  more  rarely,  tuberculous 
myelitis  occurs  from  direct  extension,  usually  through  the  lymphatic 
channels. 

Symptoms. — The  chief  symptoms  are  pain  in  the  back,  stiffness  of 
muscles,  rigidity  and  tenderness  on  motion  in  the  early  stages.  With 
the  development  of  a  kyphosis,  lordosis  or  scoliosis,  deformities  appear 
and  symptoms  of  cord  compression  and  plexus  pressure  begin.  The 
plexus  symptoms  vary  with  the  segments  involved,  as  already  dis- 
cussed. (See  Peripheral  Plexus  Neuritides.)  The  cord  compression 
gives  rise  to  increasing  bilateral  spasticity  below  the  site  of  the  lesion, 
slowly  developing  sensory  impairment,  which  may  develop  into  signs 
of  a  complete  cutting  off  of  the  entire  cord  pathways  below  the  site 
of  the  compression.     (See  table,  p.  343.) 

Lymphocytosis  in  the  cerebrospinal  fluid  is  a  frequent  sign  and  the 
cell-picture  is  striking.  X-ray  examination  will  reveal  early  the  signs 
of  tuberculous  bony  disease. 

The  symptoms  usually  show  very  gradually,  as  the  tuberculous 
disease  is  usually  a  slow  one.  Occasionally  they  show  a  fulminating 
course,  especially  in  childhood,  or  even  in  young  adults. 

The  outcome  depends  upon  the  success  obtained  in  combating  the 
tuberculosis  by  orthopedic,  surgical  and  general  health  measures. 
Early  diagnosis  is  essential,  and  the  spinal  fluid  examinations  and 
a:-ray  findings  will  aid  in  such  an  early  sizing  up  of  the  situation, 
which  will  lead  to  the  proper  procedures.  Surgical  therapy,  early, 
Albee's  bone  splint  or  related  procedure  may  help  to  do  away  with 
the  cumbersome  braces  formerly  so  extensively  used  in  treating 
Pott's  disease. 

Spinal  Cord  Tumors. — The  consideration  of  spinal  cord  tumors 
makes  a  large  chapter  in  contemporary  neurology,  which  can  only 
be  sketched  here.  They  are  comparatively  rare,  yet  frequent  enough 
to  put  one  on  one's  guard  in  any  spinal  cord  condition  showing  com- 
pression phenomena,  i.  e.,  weakness,  spasticity,  and  increased  reflexes, 
i.  e.,  pressure  symptoms  of  the  spinal  motor  neuron. 

Spinal  cord  tumors  are  as  variable  as  those  found  within  the  cranial 
cavity.  They  are  found  extradurally,  durally,  intradurally  and 
intramedullary.  They  are  small  and  large,  and  located  at  any, 
sometimes  at  all,  levels  (multiple  sarcomata)  of  the  cord.  The  tumors 
of  the  spinal  cord  are  identical  with  those  of  the  brain  {q.  v.).  They 
induce  different  symptoms  solely  because  their  localization  varies. 
For  the  most  part  they  lie  laterally  and  posteriorly,  and  are  thus  more 
accessible  to  surgical  removal.  They  occur  at  all  ages,  and  in  both 
sexes,  and  show  a  frequency  comparable  to  those  located  in  the 
cranial  cavity.  The  causes  for  intracranial  tumors  apply  to  those 
of  the  vertebral  cavity. 

Symptoms. — The  chief  symptoms  of  spinal  cord  tumor  are  pains, 

sensory  signs  on  the  skin  of  the  body,  and  evidences  of  motor  paresis. 

Sharply  localized  pains  at  the  site  of  the  tumor  are  frequent,  but  may 


350 


LESIONS  OF   THE  SPINAL  CORD 


be  absent;  all  pains  may  be  absent,  but  this  is  infrequent.^  The 
more  usual  pains  are  those  of  the  root  area  or  areas  involved  by  the 
tumor.  The  pains  are  usually  unilateral,  but  widespreading  tumors 
cause  bilateral  pain  later.     Any  so-called   neuralgia,   sciatica,   etc., 

may  be  the  initial  pressure  pain  of 
a  spinal  cord  tumor  (see  discussion 
on  neuralgias).  Under  the  popu- 
lar misnomer, "  rheumatism,"  many 
spinal  cord  tumors  and  neuralgic 
pains  are  hidden  until  it  is  too 
late  to  obtain  relief. 

The  .r-ray  picture  is  usually  nega- 
tive. The  cell  count  of  the  cerebro- 
spinal fluid  often  gives  much  infor- 
mation, and  most  tumors  show  a 
high  globulin  content  in  the  cere- 
brospinal fluid.  The  Wassermann 
technique  will  rule  out  gummata 
as  a  rule.  In  some  tumors  there 
is  an  absence  of  fluid,  or  fluid 
under  a  very  low  pressure,  below 
the  site  of  the  tumor,  with  normal 
fluid  above. 

The  motor  compression  signs 
are  variable,  paresis  advancing  to 
paralysis  (paraplegic),  hypertonic- 
ity,  spasticity,  increased  reflexes, 
Babinski,  clonus,  etc.  At  the  l6vel 
of  the  tumor  there  may  be  de- 
structive lesions  with  signs  of  per- 
ipheral motor  neuron  disease,  i.  e., 
atrophy,  loss  of  reflexes  in  the  dis- 
eased area,  reaction  of  degenera- 
tion, trophic  changes  (bed-sores, 
etc.). 

The  sensory  phenomena  will  vary 
also,  one  side  often  showing  more 
markedly  than  the  other.  There 
is  hyperesthesia  at  about  the  level 
of  the  lesion  which  is  changed  to 
various  grades  of  anesthesia  below 
the  lesion.  Slight  loss  of  epicritic 
touch  is  apt  to  be  an  early  sign. 
Greater  sensory  loss  advances  with  increasing  compression.  Bladder 
and  rectal  disturbances  are  frequent. 


Fig.  182. 


-Osteophytes  of  spinal  cord. 
(Larkin.) 


Bailey,  Jour.  Amer.  Med.  Assoc,  1914. 


LATERAL  SCLEROSIS  GROUP  351 

Small  tumors  (cysts, etc.),  intramedullary,  sometimes  extramedullary, 
cause  typical  dissociation  symptoms  with  retention  of  epicritic  tactile 
sensibility  and  loss  of  protopathic  pain  and  thermal  sensibility. 

The  symptoms  for  localization  of  the  tumor  have  already  been 
discussed. 

Diagnosis.  — -  X-ray  examination,  spinal  fluid  examination,  and 
a  complete  neurological  status  should  enable  one  to  arrive  at  a  satis- 
factory diagnosis.  The  chief  diagnostic  problem  is  syphilitic  meningo- 
myelitis.  Bony  disease  is  usually  excluded  by  the  x-rays.  Multiple 
sclerosis  is  not  infrequently  ushered  in  with  sensory  signs,  also  syringo- 
myelia. They  present  real  difficulties  in  diagnosis.  The  application 
of  proper  neurological  and  psychoanalytic  procedures  will  exclude  the 
conversion  symptoms  of  hysteria. 

Intramedullary  or  extramedullary  diagnosis  is  practically  unim- 
portant since  spinal  cord  surgery  has  become  so  satisfactory.  Theoret- 
ically employed  the  problem  is  of  interest,  practically  less  so.  For 
localization  symptoms  consult  Figs.  177,  178,  179,  and  Plates  X 
and  XL 

Treatment. — Apart  from  syphilis  the  treatment  is  surgical  and  it 
should  be  employed  early  and  for  practically  any  or  all  tumors;  not 
necessarily  with  the  promise  of  cure  but  as  a  routine  exploratory  pro- 
cedure. In  skilled  hands  the  danger  is  slight,  and  apart  from  special 
contra-indications,  the  risk  is  very  frequently  worth  while,  as  very 
unexpected  things  turn  up  within  the  spinal  cavity. 

The  diagnosis  for  localization  is  the  most  difficult  problem.  Most 
tumors  are  found  a  couple  of  inches  above  the  site  usually  sought  for 
as  determined  by  the  level  of  the  anesthesia.  Surgical  progress 
relative  to  spinal  cord  tumors  is  rapidly  advancing  and  cannot  be 
discussed  here.  The  general  prognosis  has  improved  both  with  refer- 
ence to  the  finding,  as  well  as  the  successful  removal  of  the  tumors. 
Recurrences  do  occur,  and  many  patients  are  not  benefited.  Little 
is  to  be  expected  in  those  long-standing  tumor  cases  with  marked  signs 
of  spinal  cord-compression  degeneration.  Yet  every  patient  is  entitled 
to  the  benefit  of  the  doubt.  Surgical  failure  is  better  than  a  let-alone 
policy  covered  up  by  morphinism.  This  latter  even  is  not  necessary, 
since  careful  division  of  the  affected  sensory  roots  in  inoperable  cases 
may  give  relief  from  pain,  even  if  life  cannot  be  saved. 

Most  patients  die  of  the  myelitis  bed-sore  and  bladder  complications 
after  one  to  two  years  in  inoperable  cases. 


LATERAL  SCLEROSIS  GROUP. 

Historical. — These  disorders  which  clinically  may  resemble  a  number 
of  spinal  cord  conditions,  have  been  isolated  from  the  mass  of  rachial- 
gias  of  the  eighteenth  century,  and  from  the  paraplegias  and  myelitides 
of  the  nineteenth  more  particularly  by  Seguin,  Tiirck  and  Erb.     The 


352  LESIONS  OF   THE  SPINAL  CORD 

latter,  in  1875/  posited  a  hypothetical  degeneration  of  the  pyramidal 
tracts  in  their  spinal  course,  terming  the  disease  primary  spastic 
paralysis.  Charcot  accepted  Erb's  teachings  and  spoke  of  the  dis- 
turbance as  a  spasmodic  tabes  dorsalis.  The  disorder,  as  understood 
by  Erb,  has  been  found  to  be  much  less  frequent  than  was  at  first 
supposed,  as  the  lateral  sclerosis  picture  was  found  to  be  but  one  stage 
of  a  number  of  other  cerebral  and  spinal  affections,  notably,  as  in 
multiple  sclerosis,  syringomyelia,  hydrocephalus,  tumor  of  the  cord, 
anemia  of  the  cord,  diffuse  myelitis,  senile  changes  and  amyotrophic 
lateral  sclerosis,  etc.  Of  recent  summaries,  those  of  Erb^  and  Spiller^ 
are  available. 

Pathology. — Ideally  this  consists  of  a  simple  degeneration  of  the 
pyramidal  tracts  which  rarely  ascends  to  the  cortex.  A  replacement 
glia  infiltration  is  present.  But  few  autopsies  are  recorded  with  the 
ideal  lesion. 

Symptoms. — ^These  are  exclusively  motor,  and  usually  of  the  lower 
extremities  alone,  although  the  disorder  may  show  itself  in  the  arms. 
The  ordinary  picture  is  that  of  a  spastic  paraplegia,  i.  e.,  motor  weak- 
ness, increased  reflexes,  clonus,  Babinski,  spasticity,  with  no  sensory 
or  visceral  signs. 

Gradually  increasing  weakness  of  one  or  both  legs  is  first  observed 
after  a  long  walk,  or  dancing,  or  any  prolonged  muscular  exertion. 
A  certain  stiffness  develops,  but  very  slowly,  often  only  after  several 
years.     The  tendon  reflexes  are  exaggerated  at  a  very  early  date. 

Slight  motor  weakness  supervenes,  and  some  stiffness  to  passive 
motion,  and  then  the  patients  note  that  their  gait  is  more  constrained; 
they  do  not  bend  their  knees,  but  shuffle  somewhat  and  stumble 
easily.  The  limbs  become  stiffer  and  stiffer,  the  extensor  muscles 
being  more  involved,  this  brings  about  extreme  rigidity  at  the  joints. 

In  this  stage,  which  may  be  arrived  at  in  a  few  years,  or  more  often 
after  many,  the  patient  walks  with  a  stiff,  stubbing  tread,  perhaps 
the  knees  overlap  (scissors  walk)  or  knock  each  other;  the  toes  are  not 
lifted,  but  rather  shoved  along,  thus  wearing  the  shoes  markedly  at 
the  toe. 

The  cutaneous  and  tendon  reflexes  are  exaggerated.  There  is 
markedly  increased  knee-jerk  (see  Franz's  distinctions),  patellar 
clonus,  ankle-clonus,  Babinski,  and  paradoxical  and  Bechterew- 
Mendel  signs,  Striimpell's  tibialis  phenomenon  is  apt  to  be  marked. 
Spasms  frequency  occur  in  the  affected  limbs.  In  the  latest  stages 
flexor  contractures  usually  result. 

Sensory  symptoms,  save  the  muscular  pains  of  spasm,  are  absent. 
The  bladder  and  rectum  are  not  implicated.  In  rare  instances  the 
upper  extremities  are  involved,  and  even  the  medullary  and  bulbar 
motor  tracts. 

1  Virchows  Arch.,  vol.  Ixx. 

2  Deut.  Zeits.  f.  Nhk.,  1903,  No.  23. 

3  Osier's  Modern  Medicine,  vol.  vii;  Bono,  Rev.  d.  Med.,  March,  1908. 


COMBINED  SCLEROSES  353 

Forms  and  Varieties. — 1.  Hereditary  Familial  Types  (Striimpell/ 
Newmark"). — Here  the  disorder  shows  itself  either  in  childhood,  or 
late  in  life,  and  many  generations  may  be  affected. 

The  symptoms  are  those  of  muscular  hypertonia,  spasticity,  exag- 
gerated tendon  reflexes,  and  later  palsies  and  contractures.  The 
skin  and  anal  reflexes  are  less  apt  to  be  involved  in  the  familial  types. 
In  some  cases  (Newmark,  Lorrain)  there  are  more  widespread  disorders, 
optic  atrophy,  feeble-mindedness,  muscular  atrophies,  etc.  Deep 
sensibility  is  occasionally  involved  with  slight  Romberg. 

The  changes  in  these  patients  tend  to  become  more  or  less  stationary 
after  a  certain  length  of  time,^  but  the  disease  may  extend  to  the  upper 
part  of  the  cerebrospinal  axis. 

2.  Infantile  Types. — Infantile  types  beginning  from  three  to  six 
years,  due  to  developmental  defect  in  the  pyramidal  system.  Here 
the  lower  extremities  are  most  involved.  The  advent  of  the  Wasser- 
mann  technique  is  relegating  the  greater  number  of  these  to  some 
intra-uterine  or  early  syphilitic  process. 

3.  Unilateral  Ascending  and  Descending  Types.'^ — ^The  validity  of 
this  type  is  not  yet  established.  It  shows  itself  as  a  gradually  pro- 
gressive hemiplegia,  supposedly  due  to  primary  degeneration  of  the 
pyramidal  tracts. 

4.  Mixed  Types. — These  previously  described  as  due  to  syphilis, 
to  lead,  to  lathy rus  poisoning,  anemia,  etc.,  are  more  properly 
more  or  less  irregular  forms  of  myelitis,  and  are  treated  under  that  title. 

5.  Congenital  Type  (Little's  Disease). — This  will  be  considered  under 
diseases  of  the  brain.  The  pyramidal  tract  disease  is  secondary  to 
other  lesions. 

Diagnosis. — Pure  types  of  lateral  sclerosis  are  rare.  The  underlying 
condition  often  develops  after  careful  observation,  sometimes  extended 
over  several  years.  The  chief  disorders  to  bear  in  mind  are  multiple 
sclerosis,  compression  from  tumor,  bone,  amyotrophic  lateral  sclerosis, 
myelitis,  old  encephalitis,  brain  tumor,  with  or  without  hydrocephalus. 

Hysteria  can  be  readily  excluded  by  the  careful  scrutiny  of  the 
reflexes  (Babinski,  Grasset,  Hoover  signs,  etc.). 

Treatment. — Foerster's  operation,  or  posterior  root  section,  may 
help  the  spasticities  in  some  patients.  At  the  present  time,  nothing 
is  known  that  will  stop  the  advance  of  the  disorder.  A  Wassermann 
examination  should  be  made  in  all  cases,  as  in  some  an  unknown 
syphilitic  element  has  been  revealed. 

COMBINED  SCLEROSES.     COMBINED  DEGENERATIONS. 

One  speaks  didactically  of  a  combined  sclerosis  as  a  system  disease, 
in  which  the  lateral  and  posterior  columns  are  involved;  clinically 

1  Arch.  f.  Psych.,  x,  xvii,  1880.  2  Deut.   Zeits.   f.   N.,   27,   1904. 

3  Bono,  Rev.  d.  Med.,  March,  1908,  for  summary  of  all  cases. 

4  Mills-Spill er,  Bull.  Univ.  Penn.,  1906. 

23 


354  LESIONS  OF   THE  SPINAL  CORD 

a  combination  of  tabes  and  lateral  sclerosis.  It  is  a  doubtful  question 
whether  any  one  disease  exists  which  may  be  designated  as  a  combined 
sclerosis,  but  one  does  find  a  great  number  of  conditions  in  which  both 
lateral  and  posterior  columns  are  degenerated  to  a  greater  or  less 
extent,  and  as  a  result  one  gets  varying  symptom-pictures  as  the  one 
or  the  other  is  more  or  less  implicated. 

Thus  in  true  tabes,  degeneration  of  the  lateral  columns  not  infre- 
quently occurs;  in  general  paresis  there  are  typical  combined  scleroses; 
in  many  cases  of  syphilitic  meningomyelitis,  degenerations  of  posterior 
and  lateral  columns  occur,  likewise  in  severe  anemias,  in  poisoning 
from  lead,  ergot,  pellagra,  lathyrus;  in  the  senile  cord  similar  changes 
are  found.  In  fact  a  great  variety  of  degenerations  are  found  in  the 
cord  involving  both  sets  of  columns  to  a  greater  or  less  extent.  Sand 
proposes  to  divide  this  group  into  the  pseudosystem  diseases  and  the 
polysystem  diseases.^ 

Whether,  as  Westphal  first  maintained  (1867),  there  is  a  true 
system  disease  of  these  columns  is  not  yet  a  settled  question.  Among 
this  large  group,  however,  certain  clinical  types  stand  out,  which  permit 
of  more  or  less  clear-cut  description.  In  practice  they  are  separated 
one  from  another  only  with  great  difficulty.  By  Leyden  and  his 
school,  most  of  these  disorders  were  classed  with  the  chronic  myeli- 
tides. Henneberg^  uses  the  term  funicular  myelitis  for  one  group  of 
non-system  combined  degenerations.  These  latter  are  usually  due  to 
blood  changes,  and  are  discussed  in  this  volume  with  the  myelitides 
rather  than  with  the  combined  scleroses. 

The  more  fixed  of  the  combined  scleroses  types  which  are  here 
considered  are:  (1)  combined  sclerosis  (ataxic  paraplegia  (Westphal) ), 
and  spastic  paraplegia  (Striimpell)  forms;  (2)  general  paresis  forms 
(see  under  General  Paresis);  (3)  toxic  forms;  (4)  senile  forms.  There 
are  many  intermediary  forms. 

1.  Combined  Sclerosis  (Ataxic  Paraplegia  (Westphal)  Types). — Here 
the  characteristic  features  are  those  of  a  spinal  tabes,  with  some 
signs  of  spasticity,  i.  e.,  Babinski's  reflex,  and  a  crawling  rather  than  a 
typical  tabetic  gait.  Ataxia,  pains,  bladder  disturbances,  sometimes 
pupillary  stiffness,  etc.,  indicate  that  the  meningoneuritic  element  is 
predominant,  the  pyramidal  tract  involvement  of  less  marked  extent. 
Certain  patients  start  with  typical  ataxic  signs,  then  gradually  develop 
spasticities  and  the  spastic  element  finally  becomes  predominant. 

Spastic  Ataxic  Type. — Here  the  spastic  element  enters  predominantly 
into  the  picture.  Weakness  precedes,  the  gait  then  becomes  stiff,  and 
the  toes  drag;  there  are  increased  skin  and  tendon  reflexes,  just  as 
in  atypical  lateral  sclerosis;  then  pains  develop;  radicular  sensory 
disturbances  commence;  the  knee-jerks  become  diminished;  hypotonia 
gradually  takes  the  place  of  hypertonia;  one  leg  may  be  hypotonic, 
the  other  hypertonic;  visceral  disorders  are  added,  and  ataxia  and 

1  Bull,  de  I'Acad.  Roy.  de  Med.  de  Belg.,  1903.  «  Arch.  f.  P.,  40,  1905. 


SYRINGOMYELIA  355 

Romberg  are  present.  Possibly  there  is  added  optic  atrophy,  or 
pupillary  stiffness.  Nystagmus  is  not  infrequent.  The  Babinski 
reflex  is  apt  to  persist. 

The  course  in  both  forms,  of  which  there  are  all  possible  gradations, 
is  chronic.  Complete  disuse  of  the  lower  limbs  follows.  The  patients 
are  bed-ridden  with  contracted,  drawn-up  limbs.  Twitchings,  spasms, 
and  bed-sores  supervene. 

2.  Combined  Sclerosis  in  Paresis. — (See  Paresis.) 

3.  Toxic  Forms. — Here  one  may  group  a  motley  array  of  combined 
scleroses.  These  are  due  to  poisoning  from  the  lepra  bacillus,  to 
diabetes,  to  lathyrus,  to  pellagra,  to  ergot,  to  alcohol,  carcinosis, 
malaria,  tuberculosis,  Addison's  disease,  etc.^ 

4.  Senile  Forms. — Slowly  progressing  weakness  of  the  limbs,  with 
numbness,  palsies,  and  stiffness  is  frequent  in  many  old  people. 
There  develops  a  shuffling  gait,  and  gradually  a  more  or  less  complete 
paraplegia  with  increased  reflexes,  Babinski  and  clonus.  The  upper 
extremities  share  somewhat  in  the  feebleness,  tremor  and  spasticity. 
These  senile  myelopathies  are  very  diverse  in  their  nature.  L'Her- 
mitte^  finds  (1)  perivascular  sclerosis,  (2)  marginal  scleroses,  and  (3) 
combined  scleroses  of  the  pyramidal  and  posterior  tracts.  Crouzon 
has  found  in  this  last  group,  paretospasmodic,  ataxospasmodic,  and 
ataxo-cerebello-spasmodic  types.  Thus  it  may  be  seen  that  the  senile 
cord  offers  a  great  variety  of  pathological  changes  with  a  large  series 
of  closely  related  clinical  pictures. 

SYRINGOMYELIA. 

Historical. — As  long  ago  as  1564,  Etienne  described  cavity  formation 
in  the  spinal  cord;  it  was  further  recorded  a  hundred  years  later  by 
Bonet  (1688)  in  his  celebrated  Sepidchretum.  Morgagni  (1740)  and 
Portal  (1800)  saw  and  described  cases,  and  Ollivier  of  Anglers  (1834) 
first  gave  the  name  syringomyelia  to  what  had  been  taught  by  Etienne 
to  be  a  persisting  central  canal,  but  which  Ollivier  claimed  was  a  patho- 
logical formation. 

The  studies  of  Gull,  of  Landau,  and  Nonat,  with  those  of  Stilling 
and  Waldeyer,  first  gave  the  impetus  to  the  correlation  of  the  ana- 
tomical lesion,  and  the  clinical  symptoms..  Duchenne  (in  1853)  then 
called  attention  to  certain  muscular  atrophies  with  sensory  anomalies, 
which  differed  markedly  from  the  chronic  muscular  atrophies  he  was 
describing;  but  it  was  not  until  as  late  as  1882  that  both  Kahler  and 
Schultze  brought  out  the  factors  that  permitted  a  diagnosis  during  life. 

This  marks  the  period  of  active  neurological  interest  in  the  disease 
with  a  sudden  growth  in  its  symptomatology.  Wichmann  (1887)  and 
Anna  Baumler  (1888)  published  monographs,  the  latter  collecting 
112  cases. 

1  For  literature  to  1903,  see  Sand,  1.  c. 

2  Thfese  de  Paris,  1907, 


356  LESIONS  OF   THE  SPINAL  CORD 

In  the  six  years  following,  up  to  the  appearance  of  the  first  edition 
of  Schlesinger's  masterly  monograph,  contributions  appeared  from 
everywhere,  and  the  related  subjects  of  leprosy  and  Morvan's  disease 
took  on  an  active  interest.  Schlesinger's  (1894)  monograph  fixed  the 
lines  of  research,  which  are  shown  in  his  thorough  and  monumental 
second  edition  of  1902,  a  monograph  of  some  600  pages,  since  which 
time  few  striking  additions  have  been  made. 

Etiology. — Men  are  more  frequently  affected  than  women,  in  about 
the  proportion  of  2  to  1.  About  70  per  cent,  of  the  cases  occur  before 
the  age  of  forty,  the  greatest  age  of  incidence  being  between  the  ages 
of  twenty  and  thirty.  It  is  not  known  that  occupation  has  any  bear- 
ing on  the  etiology.  Toxic  factors  are  not  proved;  infections  may 
play  a  role  in  causing  cord  hemorrhages,  emboli  or  thrombi,  with 
secondary  cavity  formation — Schlesinger  believes  that  in  such  develop- 
ments the  cord  was  not  previously  normal.  Syphilis  may  be  such  an 
infecting  agent,  also  the  typhoid  bacillus.  Syringomyelia  in  mother 
and  son,  and  in  different  menibers  of  the  same  family  has  been 
observed,  but  Schlesinger  did  not  note  any  hereditary  history  in  any 
of  his  numerous  cases. 

Traumatism  undoubtedly  plays  a  role.  Hematomyelia  develops 
after  spinal  traumata,  and  then  may  give  rise  to  secondary  cavity 
formation.  Kienboch,  however,  has  followed  many  of  these  cases  of 
trauma  and  rarely  found  any  consequent  syringomyelia.  Local  trau- 
mata with  ascending  neuritis  have  possibly  given  rise  to  later  developing 
syringomyelia.    Leprosy  may  also  condition  a  syringomyelia. 

The  essential  feature  is  a  congenital  predisposition.  The  various 
factors  just  enumerated  are  purely  contributory,  either  singly  or  one 
or  more  together. 

Symptoms. — Like  multiple  sclerosis,  syringomyelia  is  characterized 
by  its  extremely  rich  and  variable  symptomatology;  like  this  disorder 
also,  almost  anything  is  to  be  expected,  since  with  the  gradual 
increase  in  extension  of  a  cavity  formation  in  the  cord,  new  areas 
become  involved,  and  older  boundaries  enlarged.  It  is  therefore  a 
disorder  which  is  apt  to  show  a  gradually  developing  symptomatology, 
and  persisting  hs  it  does  many  years,  a  rich  array  of  pictures  may 
be  presented.  Again  it  is  very  irregular  in  its  manifestations,  although 
certain  features  form  the  background  of  the  disorder.  These  chief 
features  are:  (1)  a  peculiar  dissociation  of  the  sensory  impulses;  (2) 
muscular  atrophy  and  other  trophic  disturbances,  affecting  chiefly  the 
skin  and  joints;  (3)  motor  disturbances  either  irritative  or  paretic  in 
character.  Not  all  of  the  cases  show  these  symptoms,  but  inasmuch 
as  the  cavity  formation  is  apt  to  occupy  certain  portions  of  the  cord 
more  often  than  others,  this  grouping  of  symptoms  occurs  more  often 
than  other  groupings. 

1.  Sensory  Dissociation  (Kahler,  Schultze). — This  consists  in  a  loss 
of  ability  to  recognize  sensations  of  heat  and  cold  and  sensations  of 
pain  but  without  any  loss  of  touch,  especially  of  epicritic  touch. 


SYRINGOMYELIA 


357 


This  dissociation  varies  considerably.  It  may  be  absent.  In  one 
patient  or  in  one  area  the  thermo-anesthesia  is  very  profound,  the  loss 
of  pain  sense  less  so — again,  in  others,  the  reverse  is  true.     In  the 


Fig.   183. — SjTingomyelic  cavity  in  the  cord  (cervical  region). 


Fig.   184. — Another  level  of  the  cord,  showing  gliomatous  tumor  ca\'ity. 


majority  of  patients  the  loss  is  only  a  partial  one,  not  an  absolute  one. 
Most  cases  if  seen  early  enough  will  show  only  slight  reductions — 
epicritic  heat  and  cold  are  lost  before  protopathic  heat  and  cold — 


358 


LESIONS  OF  THE  SPINAL  CORD 


sometimes  the  reverse  is  true,  or  thermo-anesthesia  and  analgesia 
may  be  present  on  one  side  only.  Again  heat  or  cold  alone  may  be 
affected.    Epicritic  touch  may  be  involved,  but  it  is  not  usual. 

These  sensory  anomalies  ma^'^  be  distributed  over  very  small  areas, 
rarely  bulbous  (mucous  surfaces  included),  most  often  cervical;  or 
they  may  extend  almost  throughout  the  entire  spinal  axis  from  the 
trigeminus  to  the  cauda  (loss  of  testicle  pains,  and  analgesia  of  the 
bladder,  etc.). 


Fig.  185.- 


-Syringomyelia,  showing  the  dissociated  loss  of  sensibility, 
sensibility  to  pain. 


Areas  of  loss  of 


The  areas  of  diminished  or  lost  sensibility  to  heat  and  pain  are 
usually  bilaterally  asymmetrical,  not  infrequently  are  they  unilateral 
for  a  time,  then  spread  to  the  opposite  side.  They  show  the  most 
unique  distributions.  The  distribution  may  be  exquisitely  radicular; 
again,  it  is  predominantly  segmental  or  metameric.  Schlesinger's 
most  recent  researches  speak  for  the  segmental  type  of  distribution 
for  the  majority  of  the  cases  (Fig.  185). 


SYRINGOMYELIA 


359 


In  the  beginning,  one  frequently  encounters  the  glove  and  stocking 
types  of  sensory  changes.  Later  a  whole  limb  will  be  involved;  one 
may  get  a  girdle  sensation.  A  hemi analgesia,  or  hemithermo-anes- 
thesia  may  be  present.     Allochiria  is  usually  absent. 

The  sensory  changes  usually  take  years  to  develop.  This  sensory 
dissociation  is  frequently  preceded  by  paresthesise — burning  pains, 
cold  spots,  neuralgic-like  pains,  etc.  Position  sense  is  rarely  involved, 
even  in  the  advanced  cases.  Tactile  agnosia  (astereognosis)  is  frequent. 
The  bony  sensibility  is  very  frequently  involved  (Egger).  It  may  also 
be,  although  rarely,  an  initial  sign.  Deep  pressure  sense  is  often 
involved.     Romberg  and  ataxia  are  naturally  not  infrequent. 

2.  Muscular  Atrophies  and  Other  Trophic  Changes. — These  changes 
occur  most  often  in  the  small  muscles  of  the  hand  (ulnar  distribution), 


Fig.  186. — Syringomyelia  ("prediger  hande)." 


and  of  the  arms.  Thus  are  produced  typical  claw-hands,  which  develop 
slowly  and  insidiously.  Here  again  no  absolute  rule  is  followed.  The 
interossei  may  atrophy  first,  or  the  muscles  of  the  thenar  or  hypo- 
thenar  eminences.  A  (median)  typical  monkey  atrophic  hand  may  be 
present,  or  the  "preacher-hand."  Or  the  atrophy  in  the  hands  may  be 
skipped,  or  combined  with  that  of  the  shoulder-girdle  (Aran-Duchenne 
types),  the  wings  of  the  scapula  stand  out,  etc.  Atrophy  of  the 
trunk  muscles  results  in  various  distortions  of  the  spinal  column. 
Pes  equinus,  pes  valgus,  etc.,  occur  in  the  lower  extremity,  though  less 
frequently.  The  bulbar  neuclei  may  be  involved,  causing  speech  dis- 
turbances; and  these  may  be  initial  symptoms,  though  infrequent. 

These  atrophies  are  usually  progressive;  like  the  sensory  changes, 
they  are  usually  bilaterally  asymmetrical,  and  show  much  variability. 


360 


LESIONS  OF   THE  SPINAL  CORD 


Thus  one  may  find  a  claw-hand  on  one  side,  and  a  monkey-hand  on 
the  other,  etc. 

Reaction  of  degeneration  varies,  being  present  in  some  and  not  in 
others.    Increase  of  electrical  excitability  is  present  in  others. 

Fibrillary  twitchings  are  common  in  the  degenerating  muscles,  and 
even  more  active  movements  are  observed,  consisting  in  static  tremors, 
choreic-like  movements,  intention  tremors,  or  paralysis  agitans-like 
tremors.  Cramps  are  not  infrequent,  and  peculiar  myotonic  contrac- 
tions. These  irritative  motor  phenomena  belong  more  to  the  early 
stages. 


Fig.  187. — Syringomyelia,  showing  the  curvature  of  the  back  and  the  atrophy  of  the 
small  muscles  of  the  hands. 


The  motor  power  is  uniformly  diminished  with  the  motor  atrophy, 
and  spasm  or  contractions  and  rigidity  may  be  present.  Spasticity 
is  not  infrequent  in  the  lower  extremities.  This  only  argues  for 
pressure  upon  or  involvement  of  the  pyramidal  tracts  by  the  lesion. 

The  gait  is  not  infrequently  involved.  The  patients  tire  easily; 
they  commence  to  walk  more  slowly;  hemiplegic  types,  paraplegic 
types,  patients  bent  to  one  side,  or  bent  strongly  forward  are  seen 
in  the  later  stages.  Ataxic  and  tumbling  gaits  belong  to  the  curiosities 
with  rare  bulbar  localizations.  Schlesinger  reports  a  large  variety  of 
rare  anomalies. 


SYRINGOMYELIA  361 

Trophic  Disturbances. — ^These  may  be  many.  They  may  vary  in 
number  and  extent  in  strict  accordance  with  the  involvement  of  the 
trophic  cells  in  the  cord. 

In  the  skin  one  finds  cutaneous  hyperemias,  either  active — idio- 
pathic congestive  erythemas — or  passive,  with  the  formation  of  dark 
red  patches,  or  various  patches  of  a  dark  blue  color — cyanotic.  These 
changes  are  usually  associated  with  others  of  the  muscles  or  of  the 
bones.  Cutaneous  anemias  and  cutaneous  edemas  (succulant  hand), 
with  or  without  distinct  Raynaud  symptoms,  may  occur.  The 
glandular  activities  of  the  skin  also  suffer.  The  perspiration  may  be 
absent,  or  excessive,  or  one  encounters  curious  anomalies,  such  as 
increased  perspiration  to  cold  stimuli,  or  islets  of  increased  perspiration 
in  normal  skin,  or  perspiration-absent  areas.  These  areas  of  altered 
perspiration  show  anomalous  distributions  quite  comparable  to  those 
seen  in  the  sensory  sphere.  Changes  in  the  fatty  secretions  may  be 
looked  for. 

Acute  dermatoses  are  also  encountered  with  exudative  phenomena; 
urticarias,  angioneurotic  manifestations,  phylectenulse,  dermatitis  bul- 
losse.  Hemorrhagic  infiltrations,  or  even  ulcer  and  gangrene  of  the 
skin  are  known.  True  hypertrophies,  sclerodermas,  and  changes  in 
the  nails  are  recorded. 

The  bony  structures  also  suffer.  Arthropathies  are  common  (10 
per  cent. — Sokoloff ;  25  per  cent.^ — Schlesinger),  but  more  often  in  the 
upper  extremities,  in  sharp  contrast  to  the  lower  limb  distribution  of 
tabes.  They  usually  occur  late  in  the  disease  and  persist  for  many 
years.  The  onset  is  usually  acute,  with  pain,  swelling,  and  destruction. 
Mild  cases  cause  little  or  no  deformity.  Both  atrophic  and  hyper- 
trophic changes  occur,  with  fixation  of  the  joints,  dislocations  or  fract- 
ures. The  sensory,  secretory  and  trophic  disturbances  are  usually 
in  closely  related  areas,  and  bony  sensory  anomalies  are  to  be  expected. 
Occasionally  suppurations  occur. 

Bony  changes  in  the  veretebrse,  with  thorax  deformities,  occur  either 
as  a  consequence  of  arthropathies,  or  as  muscular  atrophies.  These 
scolioses  occur  usually  in  the  upper  dorsal,  and  give  rise  to  compen- 
satory curvatures. 

The  bony  structures  of  an  entire  limb  may  be  involved — a  hand  or 
a  foot — with  atrophies  of  the  muscles.  Here  acromegaly  is  to  be  ruled 
out  usually  by  the  deformed,  contracted  nature  of  the  syringomyelic 
hand  or  foot,  the  marked  muscular  atrophy,  and  the  isolated  character 
of  the  limb  involved.  Syringobulbia  may  cause  a  facial  hemiatrophy 
or  hemihypertrophy. 

Reflexes. — Great  variability  and  changeability  is  present.  The  skin 
reflexes  vary  from  complete  loss  to  exaggeration.  The  Babinski  reflex 
may  or  may  not  be  present,  as  well  as  the  abdominal,  epigastric  and 
cremasteric  reflexes. 

The  tendon  reflexes  are  often  striking  in  that  one  reflex  in  the  arm 
for  instance  will  be  lost,  the  others  present,  or  even  exaggerated.    The 


362 


LESIONS  OF   THE  SPINAL  CORD 


Fig.  188. — Pontine  syndrome,  with  eye  palsies  of  central  origin  and  syringomyelic  dis- 
sociation. There  is  here  a  crossed  hemianesthesia  with  alternating  paralysis  of  the 
VI  and  VII  cranial  nerves,  anesthesia  of  the  V  nerve  due  to  hemorrhage  in  the  lateral 
and  lower  portion  of  the  pontine  tegmentum  of  the  left  side.  The  right-hand  figure 
shows  the  hemianesthesia,  dissociated  as  in  syringomyelia  (hemianalgesia  and  hemi- 
thermanesthesia  due  to  lesion  of  the  crossed  sensory  pathways  of  the  lateral  portion  of 
the  reticular  formation.  There  is  preservation  of  the  tactile  and  postural  sensibilities 
and  of  the  stereognostic  sense,  because  of  the  incomplete  extension  of  the  lesion  to  the 
median  lemniscus  (Rm).  The  left-hand  figure  shows  (1)  atrophic  paralysis  of  the  VII 
nerve  with  reaction  of  degeneration,  lagophthalmia,  drooping  of  the  lips,  loss  of  facial 
mimicry,  paralysis  of  the  entire  left  facial  (VII)  indicated  (Fig.  a) ;  (2)  anesthesia  of 
the  face,  following  involvement  of  the  descending  root  of  the  trigeminus  (see  V  on  a^)  ; 
(3)  paralysis  of  the  external  rectus  with  convergent  strabismus  by  reason  of  the  over- 
action  of  the  antagonists.  Furthermore,  there  is  a  paralysis  of  the  lateral  movements 
of  the  eyeballs  toward  the  left  notwithstanding  the  integrity  of  the  posterior  longitu- 
dinal fasciculus  (Flp.),  of  the  nucleus  of  the  VI  and  of  the  adjacent  reticular  formation. 
The  lesion  of  Deiters'  nucleus,  and  of  the  labyrinthine  oculorotary  fibers  which  unite 
Deiters'  nucleus  (ND)  to  the  nuclei  of  the  III  and  VI  causes  this.  By  reason  of  the  over- 
action  of  the  antagonists  the  patient  looks  to  the  right.  (After  Dejerine.)  For  abbre- 
viations of  the  anatomical  sketch  see  section  on  Midbrain. 


SYRINGOMYELIA  363 

same  holds  true  in  the  lower  extremity,  but  is  less  striking.  Increased 
reflexes  are  here  more  apt  to  be  found.  Increased  knee-jerks,  Achilles- 
jerk,  and  even  ankle-clonus  are  not  infrequent,  particularly  in  high- 
lying  syringomyelias — bulbar,  cervical  and  upper  dorsal.  Lost  knee- 
jerks,  unilateral  or  bilateral,  may  also  occur  in  syringomyelia,  either 
as  an  early  or  a  late  symptom,  and  may  be  associated  with  increased 
skin  reflexes  (Babinski). 

Rare  cases  are  encountered  with  increased  jaw  reflexes. 

Visceral  Symptoms. — Bladder  disturbances  are  not  the  rule,  although 
at  times  appearing,  usually  late  in  the  disease.  When  appearing  early 
they  are  apt  to  be  transitory.  They  may  be  sensory  or  motor,  irrita- 
tive or  paralytic.  Cystitis  is  not  infrequent  in  the  later  stages  of  the 
disease.  In  syringobulbias  one  finds  anomalies  of  secretion,  polyuria, 
glycosuria,  diabetes  insipidus,  pollakiuria. 

Obstipation  is  not  uncommon;  incontinentia  alvi  less  so.  Loss  of 
sexual  power  and  of  desire  also  occur.  Persistent  priapism  is  one  of 
the  curiosities  of  this  disorder;  also  analgesia  of  the  testicles. 

Bulbar  Symptoms.  —  These  are  frequent,  when  isolated,  giving 
rise  to  syringobulbia.  They  are  usually  more  benign  than  when 
found  in  other  affections  (chronic  bulbar  palsy),  have  a  very  chronic 
course,  are  usually  unilateral,  and  often  implicate  the  vagus.  They 
occur  in  about  one-third  of  all  cases.  Certain  affections  of  cranial 
nerves  have  been  recorded  for  many  years.  Smell  and  hearing  are 
involved  rarely.  Vestibular  vertigo  is  also  rare.  A  dulling  of  the  sense 
of  taste  is  not  infrequent,  and  shows  irregularities  of  disturbance^ — 
half-tongue  (Dejerine);  dissociation  (Hitzig,  Simon) — anteroposterior 
changes. 

Optic  atrophy  or  swelling  occurs  in  advanced  bulbar  cases;  a  few 
cases  show  restriction  of  the  color  fields.  The  eye  muscles  may 
show  nystagmus  or  nystagmoid  movements,  paralyses  and  sympa- 
thetic complications.  H.  Simon  reports  an  interesting  case  of  nystag- 
moid movements  with  both  eyes,  absent  when  only  one  was  in  use. 
Transitory  diplopias,  Schlesinger  reports  in  11  per  cent,  of  the  cases; 
they  are  not  infrequently  early  signs,  and  do  not  necessarily  recur. 
The  abducens  is  frequently  affected. 

Ptosis  is  not  infrequent,  and  is  often  an  important  early  sign.  Its 
relations  to  the  sympathetic  are  intimate.  Myosis  is  found  in  a  small 
percentage  of  cases,  and  differences  in  the  pupil  are  frequent,  at  least 
25  per  cent.  (Schlesinger).  They  are  mostly  due  to  sympathetic 
palsies.  Argyll-Robertson  pupil  has  been  observed,  but  chiefly  in  pre- 
Wassermann  times,  hence  the  absence  of  syphilis  has  not  been  proved; 
its  presence  in  syringomyelia  is  anatomically  conceivable. 

The  Klumpke  syndrome  is  frequently  met  with  and  points  to  the 
upper  dorsal  localization  of  the  lesion.  Myosis,  diminished  lid  aper- 
ture, retraction  of  the  bulbi,  and  anomalous  pupillary  reactions  (loss 
of  cocaine  dilatation,  etc.)  are  signs  of  sympathetic  involvement. 

The  trigeminus  distribution  shows   sensory   anomalies,   either  as 


364  LESIONS  OF  THE  SPINAL  CORD 

paresthesise  and  pains  (tic  douloureux)  or  as  analgesise,  with  loss  of 
the  trigeminal  reflexes  and  corneal  trophic  changes.  Dissociation  in  the 
trigeminal  distribution  is  also  observed.  Pain  and  temperature  are 
mostly  aftected.    Deep  sensibility  is  less  often  involved.    (See  Fig.  188.) 

The  facial  is  involved  in  but  few  cases  (3  per  cent.)  but  impairment 
of  ability  to  swallow  is  not  infrequent.  The  tongue  is  involved,  pro- 
jects to  the  paretic  side,  shows  fibrillary  twitching,  and  hemiatrophy. 
The  soft  palate  and  laryngeal  musculature  is  often  involved  at  the 
same  time,  which  with  the  loss  of  sensation  often  causes  deglutition 
pneumonia. 

Laryngeal  palsies  are  infrequent.  They  are  characterized  by  the 
palsy  of  one  recurrent  nerve,  and  the  usually  accompanying  homo- 
lateral palsies  of  the  pharynx  and  soft  palate.  Posticus  palsy  and 
homolateral  bulbar  palsies  go  hand  in  hand.  From  15  to  20  per  cent, 
of  all  syringomyelias  have  laryngeal  symptoms;  and  they  not  infre- 
quently antedate  the  other  bulbar  or  cranial  nerve  symptoms. 

Speech  disturbances  are  common,  as  one  or  other  of  the  related 
mechanisms  are  apt  to  be  involved.  Recurrent  palsies  give  the  high, 
rasping  voice,  tongue  involvement,  the  thick,  hot-potato  speech  of 
the  bulbar  paretic;  palatal  palsies  cause  a  "nasal"  voice — occasion- 
ally a  scanning-like  speech  is  observed. 

Tachycardias,  bradycardias,  and  dyspnea  are  among  the  rarer 
symptoms. 

Apoplectiform  or  epileptiform  attacks  have  been  described,  and  are 
sufficiently  frequent  to  attract  attention.  They,  however,  are  usually 
accompanied  by  signs  of  vestibular  involvement — rotatory  vertigo, 
nystagmus,  vomiting,  etc. 

Mental  Symptoms. — Syringomyelia  is  often  associated  with  chronic 
hydrocephalus  which  two  conditions  arise  simultaneously.  Here 
defect  symptoms  in  the  form  either  of  a  debility  or  imbecility  are 
present.  Other  patients  are  reticent,  surly  and  vindictive.  Many 
patients  come  to  develop  a  totally  perverted  feeling  about  their 
anesthesise,  analgesise,  etc.,  and  cannot  be  convinced  regarding  its 
true  character. 

Course  and  Progress. — The  great  multiplicity  of  symptoms,  and  the 
almost  fortuitous  situation  of  the  tumor  and  cavity  formation  make  it 
impossible  to  generalize  too  sharply  about  the  symptom  groupings, 
but  in  general  one  can  find  four  more  or  less  classical  types.  These 
are  the  bulbar,  the  cervical,  the  dorsolumbar,  and  the  sacrolumbar. 

1.  Bulbar  Types. — Syringobulbia. — If  restricted  solely  to  the  med- 
ullary lesions,  this  is  the  least  common  of  the  types  (Schlesinger) . 
Bulbar  symptoms,  however,  are  very  often  found  in  the  other  types. 
In  this  type  the  lesion  is  limited  to  the  cranial  nerves.  The  laryngeal 
palsies  are  prominent;  difficulties  in  swallowing  are  present,  either 
transitorally  or  persistently,  and  cause  death.  Atrophy  of  the  tongue, 
and  paresis  of  the  ocular  muscles  are  frequent.  Sensory  disturbances 
in  the  region  of  the  trigeminus  are  frequently  found. 


SYRINGOMYELIA  365 

Initial  syringobulbias,  in  contrast  to  those  developing  with  or  fol- 
lowing spinal  signs  are  not  as  dangerous  to  life  ])er  se. 

2.  Cervical  Type. — This  is  the  commonest  and  that  best  known. 
The  patients  first  complain  of  weakness  in  the  small  muscles  of  the 
hand  with  clumsiness  for  finer  movements.  Paresthesise  and  pains 
are  frequent  in  the  hands  and  arms.  The  patients  frequently  have 
severe  sores  on  the  hands  from  being  wounded  or  burned,  and  then 
note  the  advancing  analgesia,  muscular  wasting,  and  loss  of  ability  to 
tell  hot  from  cold,  anaJgesise,  total  or  partial,  with  intact  touch  sense. 
The  difficulties  are  first  unilateral,  and  later  spread  assy  metrically. 
The  knee-jerks  are  apt  to  be  exaggerated.  Advancing  disease  shows 
itself  in  the  greater  atrophy,  with  claw-hand,  preacher-hand,  monkey- 
hand,  the  whole  shoulder-girdle  may  show  involvement.  The  knee- 
jerks  are  increased,  clonus  and  Babinski  may  be  present.  There  may 
be  beginning  scoliosis  or  kyphosis.  Trophic  disturbances  in  the  upper 
extremities  appear. 

3.  Dorsolumbar  Type. — Strictly  localized  dorsolumbar  types  are 
rare.  Here  the  typical  syringomyelic  sensory  anomalies  are  met  with. 
Paresthesia,  pains  followed  by  analgesise,  thermo-anesthesia,  preserva- 
tion of  touch,  kyphoscoliosis  are  frequent.  The  muscles  of  the  pelvic 
girdle  become  involved  and  the  lower  extremities  show  atrophies  and 
deformities.  The  Klumpke  type  of  sympathetic  involvement  is  seen 
here  as  well  as  in  the  preceding  type,  i.  e.,  irregular  pupils,  irregularity 
in  the  palpebral  fissure,  recession  of  the  eyeball.  The  gait  becomes 
spastic  paretic,  the  tendon  reflexes  usually  increased — at  times  lost. 
Girdle  sensations  and  involvement  of  the  bladder  and  rectum  are  not 
infrequent.  Secretory  and  trophic  anomalies,  already  noted,  point  to 
the  localization  of  the  process.  Many  of  these  cases  closely  resemble 
tumors  of  the  cord. 

4.  Sacrolumbar  Types. — These  are  rare  types,  and  are  characterized 
by  muscle  atrophies,  especially  of  the  lower  extremities,  and  the  smaller 
muscles  of  the  feet.  The  glutei  may  also  be  involved.  Segmental 
sensory  disturbances  of  the  perineum  and  genital  regions  of  the  dis- 
sociated type  referred  to  are  present.  Trophic  disturbances  are  usually 
profound  and  extensive,  such  as  fractures,  ulcerations,  running  sores, 
etc.  Vasomotor  disturbances  are  frequent.  The  tendon  reflexes  are 
usually  increased.  Babinski  may  be  present.  Bladder  and  rectum 
are  usually  involved.  Contractures  are  common,  and  kyphoses  and 
scolioses  occur. 

Characteristic  Groupings. — Not  only  do  certain  types  stand  out 
conditioned  by  the  topography  of  the  intramedullary  lesion,  but 
certain  cases  show  predominant  forms  of  lesion,  such  as  motor,  sensory, 
trophic,  or  secretory.  Thus  certain  cases  resemble  amyotrophic  lateral 
sclerosis  very  closely;  others  again  have  the  general  features  of  a 
spastic  spinal  paraplegia,  others  again  show  a  characteristic  Aran- 
Duchenne  scapulohumeral  atrophy.  A  few  cases  of  general  anesthesia 
are  on  record,  and  certain  sensory  types  may  be  confused  with  hysteria. 


366  LESIONS  OF   THE  SPINAL  CORD 

Trophic  cases  with  an  isolated  picture  of  Morvan's  disease  are  striking, 
and  tabetic-Kke  forms  are  likewise  puzzhng.  Schlesinger  also  calls 
attention  to  a  pachymeningitic  type. 

The  illustrative  case  of  Schmitt  and  Baraban,  which  is  not  unique, 
shows  the  striking  variability  that  may  be  present  in  the  symptom- 
atology of  this  disorder.  At  various  times  this  patient  was  diag- 
nosed by  competent  authorities  as  tabes,  chronic  diffuse  myelitis, 
amyotrophic  lateral  sclerosis,  and  spastic  paraplegia  of  unknown 
causation. 

Differential  Diagnosis. — ^The  chief  disorders  that  come  in  review 
are  atypical  multiple  sclerosis,  amyotrophic  lateral  sclerosis,  tumor  of 
cord  with  spastic  paraplegia,  diffuse  sclerosis,  progressive  muscular 
atrophy,  central  myelitis,  syphilitic  meningomyelitis,  polyneuritis, 
leprosy,  pellagra  and  ergotism. 

The  course  of  the  disease  is  very  chronic.  Large  cavity  formations 
involving  the  functions  of  the  intestines  and  bladder  soon  lead  to  death 
— four  to  six  years;  whereas  if  the  lesion  does  not  compress  the  entire 
cord  and  lies  outside  of  the  more  vital  nuclei  the  patients  may  be 
followed  thirty  to  forty  years.  Dejerine  has  reported  a  case  of  fifty 
years'  development. 

The  patients  die  more  often  of  intercurrent  disease;  tuberculosis 
in  particular.  Bladder  sepsis,  with  kidney  complications,  is  also 
frequent. 

The  patients  frequently  show  an  up-and-down  course,  quite  analo- 
gous to  the  course  seen  in  multiple  sclerosis. 

Pathology. — Syringomyelia,  pathologically  speaking,  is  due  to  a 
cavity  in  the  cerebrospinal  axis  not  in  genetic  relation  to  the  central 
canal — dilatations  of  this  latter  structure  are  better  termed  hydro- 
myelias.  Cavities  due  to  hemorrhage  are  recognized  as  being  dijfferent 
from  those  in  syringomyelia  proper  and  are  classed  with  the  hemato- 
myelias.  On  opening  the  spinal  canal,  which  is  rendered  difficult  by 
reason  of  the  vertebral  deformities,  the  dura  is  usually  found  to  be 
normal;  occasionally  a  pachymeningitis  complicates  the  picture. 
The  pia  is  usually  thickened,  and  evidences  of  swelling  or  of  some 
internal  irregularity  are  seen  either  as  paths  of  grayish  degeneration,  or 
cystic-like  irregularities,  with  flattening  in  places.  The  posterior 
fissure  frequently  appears  deeply  sunken  in  the  cord,  causing,  as 
Thomas  says,  a  double-barrelled  shot-gun  appearance.  The  cord 
collapses  on  cutting  and  clear  fluid  escapes.  Repeated  section,  best 
made  after  hardening  of  the  cord,  shows  the  existence  of  one  or  several 
cavities,  of  variable  length  and  diameter,  and  occupying  various 
situations  in  the  cord.  Its  most  frequent  site  is  behind  the  neighbor- 
hood of  the  central  canal,  usually  involving  the  posterior  commissure, 
and  with  a  tendency  to  reach  backward  more  than  forward.  The  cavity 
seems  to  have  a  special  fibrous  wall,  which  is  well  limited,  smooth 
or  papillated;  often  a  gliomatous  mass  fills  the  lower  end  of  the  canal. 
In  the  hydromyelic  type  the  cavity  is  round  and  usually  occupies  the 


SYRINGOMYELIA  367 

center  of  the  cord.  The  cavity  is  lined  first  by  a  layer  of  epithelium, 
and  is  surrounded  by  a  gliomatous  wall. 

In  the  syringomyelic  type  there  are  also  some  ependymal  cells, 
but  they  are  less  regularly  arranged.  These  are  interspersed  with 
neuroglia  cells  and  rest  upon  a  solid  wall  of  glia  cells,  many  of  which 
are  in  process  of  disintegration.  Small  vessels  are  frequent,  among 
which  may  be  found  many  undergoing  hyaline  degeneration.  The 
picture  is  different  at  every  level,  and  in  many  sections  the  cavity 
has  no  lining  at  all  in  places,  bordering  directly  upon  the  nervous 
tissues.  Sections  through  the  glioma  show  perhaps  no  cavity  at  all. 
Fresh  hemorrhagic  remains  of  old  hemorrhagic  foci  are  frequent 
findings. 

The  glioma  may  be  sharply  delimited  —  central  gliosis;  or  the 
neurogliar  tissue  may  infiltrate  the  cord  in  all  directions — diffuse 
gliosis. 

Secondary  degeneration  in  the  parts  impinged  upon  or  invaded 
takes  place  by  process  of  atrophy  and  then  of  tissue  replacement 
with  characteristic  neuronophagia.  Regenerated  fibers  are  also 
encountered. 

In  cases  complicated  with  pachymeningitis  one  finds  the  lesions 
of  this  process,  and  in  the  traumatic  forms  one  usually  finds  the  remains 
of  an  ancient  fracture,  with  inflammatory  thickening  of  the  meninges 
and  pia.     The  cavity  is  usually  posterior. 

Pathogeny. — No  unanimity  of  opinion  has  yet  been  reached.  The 
general  hypotheses  are:  (1)  it  is  due  to  a  defect  of  development; 
(2)  it  is  due  to  an  intramedullary  tumor  formation  which  later  breaks 
down;  (3)  it  is  the  left-over  remains  of  an  inflammatory  (chronic 
myelitis)  or  hemorrhagic  process  (hematomyelia) ;  (4)  it  is  due  to  a 
modification  of  pressure  in  the  ependymal  canal,  brought  about  by  a 
trauma,  a  compression.  In  general  it  seems  that  no  one  hypothesis 
can  explain  all  of  the  cases. 

Thus,  according  to  Schlesinger,  one  divides  the  cavity  formations 
that  may  occur  in  the  spinal  cord  as  follows : 

1 .  Cystic  formation,  after 

(a)  traumatic  destruction  of  tissue, 

(b)  traumatic  hematomyelia, 

(c)  non-traumatic  hematomyelia. 

2.  Softening  of  an  inflammatory  or  non-inflammatory  nature  with 

short  course. 

3.  Syringomyelias: 

(a)  true  hydromyelia  (as  malformation), 

(b)  true  tumor  with  cavity  formation, 

(c)  syringomyelia  gliosis, 

(d)  syringomyelia  from  vessel  disease  without  gliosa, 

(e)  pachymeningitis  and  leptomeningitis  with  cavity  formation. 
In  a  similar  manner  the  cavity  formations  of  the  medulla  may  be 

classified  as  follows : 


368  LESIONS  OF   THE  SPINAL  CORD 

1.  Cysts  following  softening  or  hemorrhage. 

2.  Softening  of  inflammatory  nature  with  acute  course. 

3.  Cavity  formation  following  degeneration  of  tumors. 

4.  True  syringobulbias  (in  typical  localities) . 

(a)  Embryonal,  lying  in  the  center,  and  in  combination  with 

hydromyelia  or  syringomyelia. 

(b)  Without  combination  with  hydromyelia  or  syringomyelia. 

(c)  Arising  in  later  life  and  lying  laterally  as  continuation  of  a 

syringomyelia  due  to  circumscribed  bulbar  changes. 

Therapy. — Since  hemorrhage  into  the  glial  cavities  is  a  frequent 
occurrence  in  syringomyelia,  it  is  advisable  to  avoid  hard,  muscular 
work,  since  such  seems  to  favor  bleeding.  Severe  muscular  effort 
also  aids  in  spontaneous  fractures.  Occupations  involving  heat  are 
to  be  avoided  because  of  the  danger  from  burning. 

For  the  pains,  the  treatment  is  symptomatic.  Here  the  various 
analgesic  antipyretics  may  be  employed.  Aspirin,  acetanilid,  anti- 
pyrine  in  5-grain  doses  are  sufficient. 

Specific  therapy  is  as  yet  unknown.  One  is  justified  in  recommend- 
ing a  spinal  cord  operation  in  the  case  of  large  hemorrhages  with  sudden 
increase  in  pressure  symptoms.  Here  the  principle  of  open  expression 
of  the  clot  can  be  practised  to  advantage  (Elsberg). 

Certain  advantageous  results  have  been  reported  from  the  use  of 
high-frequency  currents.  These,  however,  have  not  been  employed 
long  enough  to  determine  their  absolute  efficiency.  Beaugard  and 
I'Hermitte'^  recommend  weekly  applications  of  penetration  rays,  79 
(radio  chronometer  of  Benoist),  dose  of  3  H,  at  a  distance  of  15 
cent,  between  anticathode  and  the  skin.  Twenty-six  seances  are 
recommended. 

MULTIPLE  SCLEROSIS. 

Historical. — Because  of  the  striking  variability  in  the  symptom- 
atology of  this  disorder,  it  is  not  altogether  surprising,  historically 
considered,  that  the  first  clues  to  its  final  delimitation  should  have 
been  gained  from  the  pathological  rather  than  from  the  clinical  side. 

Under  the  older  nosological  schemes  one  finds  these  patients  grouped 
as  epilepsy,  paraplegia,  tremor,  chorea,  dance  of  St.  Guy  chronica, 
and  a  host  of  other  conditions.  Although  Sylvius  de  la  Boe  recognized 
an  intention  tremor  as  different  from  other  types,  it  cannot  be  said 
that  the  conception  assumed  anything  like  its  modern  form  until  the 
work  of  Vulpian  (1862),  Ordenstein  (1868)  and  Charcot.  The  initial 
pathological  notion  was  given,  however,  by  Cruveilhier  (1832-1845) 
in  his  famous  case  of  Darges  (in  1840),  a  cook  in  the  Salpetriere,  a 
sketch  of  whose  cord  is  here  reproduced.  Carswell's  picture,  appearing 
in  1838,  is  probably  the  very  first  illustration  of  this  condition  on 
record.     From  this  time  on,  brain  and  spinal  sclerosis  became  an 

1  Sem.  Med.,  1907. 


MULTIPLE  SCLEROSIS 


369 


active  subject  of  discussion,  in  which  the  work  of  Frerichs^  and  Valen- 
tiner^  stand  out  prominently.     The  former  made  a  diagnosis  of  brain 


N 


Figs.  189  and  190.— Early  sketches  by  Carswell  and  Cruveilhier  (1838-1840)   of  the 
sclerotic  patches  in  multiple  sclerosis. 


1  Ueber  Hirnsclerose,  Arch.  f.  d.  g.  Med.  Haeser,  1849,  p.  334. 

2  Ueber  die  Sclerose  des  Gehirns  und  Riickenmark,  Deut.  Klinik,  1856,  p.  147. 

24 


370  LESIONS  OF   THE  SPINAL  CORD 

sclerosis  during  life  and  found  multiple  sclerotic  patches  at  an  autopsy, 
and  tried  to  erect  a  clinical  entity  with  differential  pathological  diagno- 
sis, which  later  was  amplified  by  his  student  Valentiner,  who  collected 
a  series  of  cases  resembling  those  of  Frerichs,  and  who  also  gave  a 
remarkable  summary  of  the  symptoms.  When  these  reports  are  read 
it  may  be  seen  that  they  would  not  be  included  within  the  modern 
concept,  yet  Valentiner  picked  out  the  facts  of  involvement  in  early 
years,  the  marked  motor  disturbances,  hemipareses,  tremors,  speech 
disturbances,  the  remissions,  the  unilateral  onset,  the  greater  involve- 
ment of  motor  than  sensory  functions,  the  involvement  of  the  cranial 
nerves,  the  long  course  and  bad  prognosis.  The  mental  symptoms 
as  outlined  by  Valentiner  are  not  as  characteristic.  A  diplegic  idiot, 
for  instance,  is  included  in  his  series  of  fifteen  cases. 

The  first  real  foundation  of  the  symptomatology  may  be  said  to 
have  been  laid  down  by  these  two  authors,  and  little  practical  progress 
was  made  until  the  work  of  Vulpian,  Ordenstein  and  Charcot  (1862- 
1869)  and  his  pupils  as  summarized  in  the  monograph  of  Bourne ville 
et  Guerard  (1869)  when  the  multiple  sclerosis  of  the  present  day 
assumed  definite  form.  As  early  as  1862,  Vulpian  and  Charcot 
brought  the  classical  triad,  scanning  speech,  nystagmus,  and  intention 
tremor  into  prominence  as  diagnostic  of  the  condition,  and  in  the 
later  studies  of  the  Charcot  school  the  clinical  and  pathological 
foundations  were  laid  much  as  they  were  held  up  to  within  recent 
times. 

It  has  been  becoming  more  and  more  evident,  however,  that  the 
picture  laid  down  by  Charcot  was  not  a  satisfactory  one.  Sensory 
pictures,  neglected  by  him,  had  to  be  reckoned  with  in  the  later  studies; 
also  interference  with  the  bladder.  Decubitus  as  a  symptom  occurs. 
Then  cerebellar  syndromes  were  recognized;  then  Erb's  spastic  par- 
alysis fell  largely  into  the  multiple  sclerosis  group,  then  this  author's 
chronic  dorsal  myelitis  with  optic  changes.  Hemiparesis  not  due 
to  embolism  or  thrombi  became  recognized.  Alternating  Millard- 
Gubler  types  were  described.  Then  many  bulbar  palsies  passed  into 
its  confines,  pontine  encephalic  pictures,  pure  ophthalmoplegias,  and 
occasional  forms  with  painful  attacks,  resembling  syphilitic  meningo- 
myelitis.  Amyotrophic  lateral  sclerosis  (Dejerine)  and  pseudo- 
paresis  were  later  diagnostic  possibilities,  showing  the  extreme  multi- 
plicity of  form  under  which  this  disorder  may  show  itself.  The  best 
recent  monograph  of  real  value  is  that  of  Miiller,  1904. 

At  the  outset  it  is  desirable  to  call  attention  to  the  fact  that  there 
exists  a  multiple  sclerosis  due  to  a  fairly  definite  pathological  process, 
and  other  clinical  types  resembling  the  former  so  clearly  as  to  be  clinic- 
ally indistinguishable,  but  in  which  one  finds  multiple  tumors,  multiple 
endarteritic  lesions  (syphilitic,  arteriosclerotic),  multiple  encephalo- 
myelitic  inflammatory  processes,  etc.  These  will  be  discussed  under 
pathology.    Whether  the  clinical  picture  to  be  described  belongs  only 


MULTIPLE  SCLEROSIS  371 

to  essential  multiple  sclerosis,  and  not  to  these  accessory  or  acci- 
dental forms  (secondary  forms — Schmaus  and  Ziegler)  is  not  yet 
definitely  known. 

Primary  multiple  sclerosis  then  is  a  disease  essentially  of  adolescent 
or  young  adults,  beginning  very  gradually,  advancing  slowly,  initially, 
very  varied,  but  ultimately  assuming  a  very  characteristic,  almost 
monotonous  character.  It  is  characterized  by  beginning  muscular 
weakness,  with  spasticity,  by  disturbances  in  speech,  nystagmus, 
intention  tremor,  by  forced  laughing  or  crying,  and  by  changes  in 
the  fundus — temporal  pallor.  Sensory  symptoms  may  be  present 
but  are  not  usually  prominent,  bladder  symptoms  are  not  uncommon, 
while  vasomotor  and  trophic  disturbances  are  comparatively  infre- 
quent. The  disease  persists  for  many  years,  has  striking  remissions — 
noted  by  Valentiner  in  1856 — and  the  patients  die  of  exhaustion  or 
intercurrent  disorder. 

Multiple  sclerosis  is  a  relatively  infrequent  disease.  Of  18,000  cases 
of  nervous  disease  at  Vanderbilt  Clinic  it  was  diagnosed  as  occurring  27 
times,  i.  e.,  a  percentage  of  0.001  per  cent.  In  European  clinics  it  would 
appear  to  be  more  frequent.  Bramwell-Williamson  show  2  per  cent. 
Possibly  better  diagnoses  account  for  these  differences,  since  the 
American  statistics  are  obtained  from  polyclinic  material.  These  are 
not  checked  by  autopsy  findings,  and  are  suggestive  rather  than 
conclusive  of  its  frequency.  Personal  figures  show  1  patient  in  200 
of  nervous  disorder,  0.5  per  cent. 

Etiology. — A  definite  position  regarding  this  cannot  yet  be  taken. 
Practically  all  of  the  infections  have  been  held  responsible,  and  it 
would  appear  that  they  stand  in  some  sort  of  causal  relationship. 
Certain  metallic  poisons,  zinc,  copper,  manganese,  give  rise  to  a  picture 
closely  resembling  multiple  sclerosis.  Trauma  has  been  held  respon- 
sible; so  also  have  sexual  excesses,  cold  and  wet  and  childbearing. 

Heredity  plays  an  important  factor,  according  to  many  (PelizeUs  and 
others),  and  Striimpell  contends  that  the  essential  feature  is  an 
abnormal  congenital  factor,  which  is  made  to  develop  by  any  one  of  the 
other  causes  here  enumerated.  Miiller's  critical  summary  would  seem 
to  exclude  practically  all  the  exogenous  causes  since  they  occur  in  but 
a  very  small  proportion  of  his  cases.  He  allows  that  a  secondary 
multiple  sclerosis,  in  the  sense  already  outlined,  may  possibly  follow 
infections,  but  that  multiple  sclerosis,  in  the  narrow  sense,  develops 
only  on  the  basis  of  a  congenital  predisposition. 

8ex  plays  practically  no  part.  Both  the  results  of  Charcot's  and 
Whitoff 's  studies,  which  spoke  for  greater  frequency  in  female  and  male 
material  respectively  are  due  to  their  particular  clientele.  Polyclinic 
statistics  nearly  always  show  a  greater  frequency  of  women,  because 
they  are  freer  to  visit  dispensaries,  and  usually  go  to  all  of  them  in  a 
big  city.  The  only  reliable  statistics  are  those  of  Miiller,  which  were 
controlled  by  autopsy.     They  show  practically  no  difference. 


372  LESIONS  OF   THE  SPINAL  CORD 

Age. — Three-fourths  of  the  carefully  observed  cases  occur  between 
the  years  of  twenty  and  forty.  In  our  own  statistics^  two-thirds  of 
the  patients  were  under  forty.  Cases  have  been  reported  in  children 
as  young  as  five  months,  and  in  adults  as  old  as  seventy-five  to  eighty 
years,  but  for  both  extremes  diagnostic  mistakes  are  not  ruled  out. 
Marie's  view  that  multiple  sclerosis  is  common  in  children  has  not 
withstood  the  severe  critique  of  autopsy  material,  and  Miiller  takes 
the  stand  that  the  disease  is  found  extremely  seldom  in  very  early 
youth.  The  initial  stages,  however,  may  be  traced  very  often  to 
the  years  of  adolescence  or  early  adult  life.  Occupation  plays  no 
role  that  is  yet  known. 

Symptoms. — The  accidental  features  of  the  distribution  of  the 
sclerotic  patches  in  this  disorder  makes  it  possible  for  almost  any 
combination  of  neurological  signs,  and  the  greater  the  number  of  cases 
studied  the  richer  has  become  the  symptomatology.  Certain  patients 
show  comparatively  few  symptoms  for  years;  others  show  additions 
almost  from  month  to  month,  until  they  become  veritable  neurological 
museums,  with  signs  of  involvement  from  the  frontal  poles  to  the 
tip  of  the  Cauda  equina.  No  two  patients  are  alike,  yet  most  seem  to 
attain  the  same  level  in  the  end  and  almost  come  to  present  a  stereo- 
typed picture. 

In  this  picture  the  most  striking  feature  is  the  involvement  of  the 
motor  system-,  giving  rise  to  muscular  weakness,  hypertonus  and 
general  spastic  phenomena,  both  in  the  cranial  and  spinal  paths. 
The  extreme  range  is  the  feature  that  characterizes  multiple  sclerosis 
in  its  fully  developed  form.  One  finds  isolated  or  group  phenomena  all 
over  the  body  just  in  the  same  proportion  as  the  accidental  distribution 
of  the  sclerotic  patches  is  isolated  or  diffuse.  For  this  reason  it  has 
been  thought  advisable  to  begin  the  description  of  the  symptoms  with 
those  of  the  cranial  nerves,  and  proceed  systematically  throughout  the 
nervous  system.  The  older  and  classical  triad  of  Charcot  is  only 
partially  true,  and  today  a  multiple  sclerosis  may  be  diagnosed  from  a 
temporal  pallor  of  the  optic  disks  combined  with  bladder  incontinence 
in  the  absence  of  nystagmus,  intention  tremor,  or  scanning  speech. 

The  classical  picture  of  Charcot  may  be  found  in  not  more  than 
15  per  cent,  of  all  the  cases,  at  a  period  when  the  presence  of  other 
symptoms  permit  one  to  make  a  diagnosis  of  multiple  sclerosis.  If 
one  waits  for  the  "classical"  picture,  one  may  have  to  wait  for 
years. 

Olfactory. — Hallucinations  of  smell  are  occasionally  found  but  belong 
to  the  rarer  and  infrequently  recorded  psychical  signs.  Bilateral 
anosmia  has  been  recorded. 

Optic. — ^The  researches  of  Uhthoff  have  emphasized  the  frequent 
occurrence  of  changes  in  the  optic  disks.  About  50  per  cent,  of  the 
patients  show  changes  in  the  papillae,  and  it  is  striking  that  these 

1  Jelliffe,  Jour,  of  Nerv.  and  Ment.  Dis.,  1904,  p.  446. 


Fig.  191 


Left  eye  visior  20/30 


Fig.  192 


Fig.  193 


Right  eye  vision  20/30 


Right  eye  vision  15/40 


Left  eye  vision  15/30  Right  eye  vision  15/50 

Fig.  194 


Left  eye  vision  20/40  Ri&^t  ^^^  ^'='°"  ^°'3° 

Field  of  vision White  -.-.-.-  Blue  Green  for  5  mm.  D 

Relative  scotoma  ^ 

Figs.   191-194. — Irregular  scotomata  seen  in  multiple  sclerosis  cases.     (Klingmann.) 


374  LESIONS  OF   THE  SPINAL  CORD 

changes  occur  early.  They  are  in  the  nature  of  partial  atrophies, 
and  show  themselves  for  the  most  part  as  a  simple  atrophic  pallor 
of  the  disk — usually  most  marked  temporally.  True  optic  atrophy 
is  found  in  some  of  the  cases,  and  again  in  still  fewer  a  true  papillo- 
edema  or  choked  disk.  This  usually  recedes  and  either  leaves  no 
sign  or  an  atrophy.     A  great  number  of  variations  are  known. 

The  sight  is  frequently  affected,  sometimes  blindness,  partial  or 
complete,  unilateral  or  bilateral,  is  an  initial  symptom.  This  not 
infrequently  clears  up.  Complete  blindness  with  double-sided  atrophy 
is  extremely  rare  (Gnanck-Uhthoff ) .  Parinaud  has  made  three 
classical  types,  but  later  studies  have  shown  that  there  is  no  uniformity, 
and  the  variations  in  sector  blindness  and  dimness  are  very  many. 
The  loss  or  diminution  in  sight  in  multiple  sclerosis  is  characterized 
by  its  advancing  and  receding  character,  and  by  the  fact  that  as  a  rule 
the  ophthalmoscopic  picture  is  usually  more  pronounced  in  severity 
than  the  symptoms,  and  offers  no  satisfactory  register  of  the  variability 
in  visual  acuity. 

The  fields  of  vision  more  often  show  central  scotomata  with  per- 
ipheral clearness  than  peripheral  scotomata.  Unilateral  scotomata, 
quadrant  scotomata,  and  a  great  variety  of  other  forms  are  known  as 
well  as  a  certain  amount  of  variability  from  time  to  time  in  the  individ- 
ual case.  The  color  fields  show  similar  variations  and  variabilities. 
Optic  hallucinations  also  occur.     (See  Figs.  191  to  194.) 

Ocular  Symptoms. — The  characteristic  phenomenon  here  is  nystag- 
mus. Its  occurrence  as  a  symptom  of  "brain  sclerosis"  was  first 
pointed  out  by  Valentiner  (1856)  and  Charcot  overvalued  its  diagnostic 
importance.  True  central  nystagmus  is  rare,  but  nystagmoid  move- 
ments on  lateral  motion  of  the  eye  occurs  in  from  70  to  80  per  cent,  of 
the  cases,  especially  in  the  later  stages  of  the  disease.  Vertical, 
oblique,  rotatory  nystagmus  is  included  with  the  other  forms.  In 
cases  with  pronounced  cerebellar  involvement  the  nystagmus  is  of 
this  type,  and  rotation  of  objects  with  subjective  rotation  and  forced 
positions  are  to  be  expected.  These  eye  movements  may  be  sum- 
marized as:  (1)  continued  rhythmical  oscillations  (true  central  nystag- 
mus) analogous  to  the  continuous  movements  of  the  head  and  body; 

(2)  rhythmic  oscillations  set  up  on  movement  of  the  bulb  in  any 
direction,  analogous  to  the  intention  tremor  of  the  hands;  (3)  nystag- 
moid movements  on  extreme  lateral  or  vertical  movements,  analogous 
to  fatigue   (paresis)   movements;    (4)    ataxic  movements.     Of  these 

(3)  is  the  most  frequent;  practically  all  are  conditioned  by  central 
disease,  although  peripheral  involvements  are  described. 

Eye  Muscle  Palsies. — These  are  important  in  diagnosis,  and  present 
themselves  infrequently  as  isolated  or  complete  palsies,  transitory  or 
persisting.  Paralysis  of  convergence  is  more  often  found.  In  general, 
from  17  to  46  per  cent,  of  the  patients  show  palsies,  the  statistics 
varying  according  to  the  more  or  less  strict  interpretation  of  paralysis. 
Uhthoff  demands  "double  vision"  as  a  criterion,  which  symptom  is 


MULTIPLE  SCLEROSIS  375 

not  an  infrequent  early  symptom  of  multiple  sclerosis.  Miiller,  on 
the  other  hand,  admits  milder  palsies  into  his  statistics  (46  per  cent.). 

Ptosis  is  less  frequently  found  (8  to  15  per  cent.),  usually  one-sided, 
occasionally  double,  usually  incomplete  and  ephemeral.  One  also 
meets  with  external  ophthalmoplegia,  abducens,  and  trochlearis 
palsies.  Divergent  palsy  has  been  noted  by  Bielschowsky.  Internal 
ophthalmoplegia  has  not  yet  been  recorded. 

Pupillary  inequalities  are  not  infrequent  (24  per  cent. — Miiller),  and 
these  are  changeable  and  frequently  early.  They  may  represent 
spasms  or  paresis  of  muscles.  Miosis  is  frequent  in  the  later  stages 
(Parinaud),  but  the  rejflexes  are  usually  normal.  Pupillary  unrest 
(hippus)  is  found  (Frankl-Hochwart) .  Argyll-Robertson  pupil  was 
found  only  four  times  in  364  cases.  One  case  of  Uhthoff  was  controlled 
by  autopsy;  two  cases  by  Rad,^  Wassermann  control. 

V.  Trigeminus. — Facial  neuralgia  has  been  observed  as  the  first 
symptom  of  a  multiple  sclerosis  (Oppenheim),  but  both  sensory  and 
motor  fifth  nerve  involvement  is  rare. 

VII.  Facial. — Facial  palsies,  usually  associated  with  other  central 
(hemiplegic)  pontine  (alternating  palsy),  or  pressure  symptoms,  occa- 
sionally coming  on  very  acutely,  are  not  infrequent  (15  per  cent.);  as 
an  isolated  palsy  it  is  rare.  Like  other  palsies  their  fluctuating  char- 
acter is  striking,  the  severe  permanent  types  being  rare  (Bouchaud), 
as  are  also  bilateral  palsies. 

VIII. — Hearing  is  rarely  affected.  Complete  or  partial  deafness 
has  been  recorded,  as  well  as  the  occurrence  of  noises.  Auditory 
hallucinations  are  possible,  but  not  anatomically  proved.  Hyper- 
esthesiee  are  frequent. 

The  vestibular  branch  of  the  eighth  nerve  is  not  infrequently  in- 
volved in  its  cerebellar  connections.  An  explanation  of  some  of  the 
more  severe  nystagmus  movements  may  be  found  here.  Giddiness 
and  dizziness  are  very  prominent  symptoms,  and  occur  either  from 
involvement  of  this  nerve  or  other  sensory  cerebral  or  spinal  tracts. 
Rotatory  giddiness  with  tendency  to  fall,  revolving  of  objects,  etc.,  is 
present  in  a  few  cases  of  multiple  sclerosis,  and  certain  Meniere  cases 
belong  here. 

Speech  Disturbances. — Charcot's  typical  speech  disturbance  had  a 
slow,  monotonous,  and  scanning  character.  This  special  type  is  present, 
however,  in  only  a  small  proportion  of  the  cases,  and  then  usually 
only  in  the  later  stages.  Miiller's  autopsy  controlled  material  (81 
cases)  gave  only  25  per  cent,  of  this  character,  whereas  patients  with 
other  types  of  speech  disturbance  run  higher — 25  to  30  per  cent. — 
making  about  half  of  all  multiple  sclerosis  cases  with  some  disorder  of 
speech.  Easy  fatigability,  with  increasing  unsteadiness;  stuttering, 
with  accentuated  mouth  movements,  are  other  types. 

Singers    soon  notice   these  alterations,  especially  the  fatigability, 

1  Neur.  Ctblatt,  1911,  xxx,  584. 


376  LESIONS  OF  THE  SPINAL  CORD 

and  an  increasing  inability  to  modulate  the  tones.  Dysarthria  and 
explosive  speech  are  obtained  in  later  stages.  Articulatory  disturbances 
with  repetition  of  syllables,  or  words,  is  frequent  in  later  stages — r,  I,  p, 
and  g  are  particularly  difficult.  Aphonias  or  other  laryngeal  complica- 
tions are  occasionally  met  with  (Rethi) .  The  tongue  occasionally  shows 
mild  transitory  palsies,  with  mild  atrophies,  usually  unilateral  and 
fibrillary  twitchings.  Ataxic  movements  of  the  tongue  are  frequent  in 
the  later  stages.  Occasionally  subjective  sensations  of  fulness,  thick- 
ness, and  difficulty  in  movement  are  observed. 

Chewing  and  swallowing  are  involved,  the  former  rarely,  the  latter 
not  infrequently,  less  often  transitorily,  as  an  early  symptom  more 
often  as  a  permanent  terminal  sign.  Excessive  salivation  seems  very 
infrequent. 

Pneumogastric  involvement  is  rare.  Miiller  reports  a  case  of 
paroxysmal  tachycardia  in  multiple  sclerosis.  Dyspnea  is  also  known, 
but  is  infrequent. 

The  taste  has  been  modified  in  a  few  cases. 

Motor  Disturbances. — The  most  characteristic  are  the  intention 
tremor,  ataxias,  and  palsies  with  later  developing  atrophies  and 
contractures. 

Ataxia. — By  the  finger-nose  test  or  the  finger-finger  test,  and  by  the 
static  position  one  can  demonstrate  in  the  upper  extremities  an  ataxia, 
separate  from  an  intention  tremor,  in  a  large  number  of  the  cases 
(Miiller,  70  per  cent.).  A  similar  ataxia  may  be  shown  in  the  lower 
extremities  in  even  a  more  striking  manner  in  testing  by  the  knee-heel 
test.  The  ataxic  movements  usually  precede  the  development  of  the 
intention  tremor,  and  are  later  often  covered  up  by  the  same.  Occa- 
sionally the  ataxias  are  increased  on  closure  of  the  eyes,  occasionally 
not,  showing  that  at  least  two  types  are  to  be  observed.  Recent  find- 
ings in  the  sphere  of  deep  sensibility,  position  sense,  etc.,  afford  an 
interpretation  of  these  ataxias.  They  vary  clinically  from  the  tabetic 
ataxia,  particularly  in  the  increased  tempo  of  the  individual  movements 
and  the  irregular  contraction  of  the  agonist  muscles  in  the  former. 
The  usual  hypertonus  of  the  multiple  sclerosis  patient  is  in  marked 
contrast  to  the  hypotonus  of  the  tabetic.  Crossed  hemiataxias  are 
recorded. 

Intention  Tremor. — Present  in  from  50  to  75  per  cent,  of  the  cases, 
this  symptom  is  one  of  the  most  striking  in  well-advanced  multiple 
sclerosis.  It  may  be  absent  for  a  number  of  years,  and  yet  a  diagnosis 
may  be  made  on  other  grounds.  When  present  it  speaks  almost  always 
for  this  disorder.  Considerable  care  must  be  exercised  in  not  confusing 
an  intention  tremor  with  an  ataxia,  and  vice  versa.  Miiller  has  laid 
considerable  stress  upon  this  point,  and  because  of  his  more  rigid  criteria 
regarding  the  differences,  states  that  real  intention  tremor  was  present 
in  only  25  per  cent,  of  his  cases.  This  intention  tremor  is  increased  by 
prolonged  effort  and  by  emotional  disturbances.  It  is  not  markedly 
increased  by  shutting  the  eyes. 


MULTIPLE  SCLEROSIS  .  377 

It  develops  gradually,  occasionally  suddenly  after  an  apoplectic 
attack,  is  more  often  bilateral,  corresponding  closely  with  the  pareses. 
The  arms  are  more  frequently  involved,  then  the  trunk,  and  then  the 
head;  rarely  the  chin  and  muscles  of  the  face. 

Only  exceptionally  are  the  legs  markedly  affected  by  an  intention 
tremor.  In  the  trunk  this  disturbance  gives  rise  to  a  type  of  con- 
tinual balancing  or  rocking  motion;  it  also  involves  the  muscles  sup- 
porting the  head,  causing  a  nodding  and  swaying  series  of  movements. 
Passive  tremors  of  the  hands  when  at  rest  are  also  observable. 

The  causes  for  the  intention  tremor  are  as  yet  unsolved,  purely 
spinal  causes  do  not  show  it,  and  the  greatest  probability  exists  in  the 
hypothesis  of  midbrain  localizations,  involving  both  incoming  sensory 
tracts  and  outgoing  motor  paths.  Similar  tremors  are  seen  in  lesions 
of  the  cerebello-rubro-thalamic  tracts.  A  complete  analysis  of  the  fiber 
tract  involvements  in  multiple  sclerosis  has  yet  to  be  made  before  the 
question  of  localization  is  settled. 

Writing  shows  characteristic  changes  due  to  both  the  intention 
tremor  and  the  ataxia.  The  strokes  are  irregular — too  thick  or  too 
thin — and  the  excursions  in  forming  the  letters  irregular. 

Motor  Power. — ^Motor  weakness  is  usually  the  earliest  symptom 
noted  in  multiple  sclerosis.  It  may  be  the  arms,  oftener  the  legs, 
or  occasionally  in  some  special  group  of  muscles;  larynx  in  singers, 
hand  and  arm  in  painters  or  players,  occasionally  bladder  or  rectum. 
Marked  fatigability  is  an  important  symptom,  and  may  precede  the 
other  symptoms  for  years. 

Spastic  paresis,  with  hypertonus,  then  develops,  and  though  varying 
considerably  in  intensity,  often  dominates  the  picture,  leading  later 
to  the  most  severe  forms  of  contractures.  The  type  may  be  hemi- 
plegic  or  paraplegic,  and  usually  indicates  involvement  of  the 
pyramidal  tracts.  The  lower  extremities  are  involved  much  oftener 
than  the  upper.  Irregular  Brown-Sequard  paralysis  is  occasionally 
present. 

Gait  disturbances  are  common  and  variable.  They  may  be  purely 
spastic,  or  in  the  cerebellar  cases,  spastic  ataxic.  Pure  ataxic  gaits 
are  very  rare.  A  spastic  paretic  gait  marks  the  pre-bed  stage. 
Occasionally  one  finds  the  tumbling  or  reeling  gait  of  cerebellar  tract 
implication. 

Certain  motor  anomalies  are  encountered  as  the  patients  rise  or 
sit  down.  A  Romberg  is  not  uncommon  on  standing,  which  is  not 
much  modified  on  closing  the  eyes. 

Muscular  Atrophies. — These  occur,  but  are  rare. 

Sensory  Phenomena. — Marie's  earlier  contention  that  sensory  dis- 
turbances do  not  belong  to  multiple  sclerosis  has  been  definitely 
disproved.^  On  the  contrary  they  are  very  frequently  found,  but 
largely  by  reason  of  their  fluctuating  character,  both  as  to  intensity 

1  Jeremias  has  collected  the  rich  literature  bearing  on  sensory  disturbances  in  multiple 
sclerosis.     Dissert.,  1898;  see  also  Miiller. 


378  LESIONS  OF  THE  SPINAL  CORD 

and  extent  are  overlooked  or  falsely  interpreted.  The  frequently  made 
false  diagnosis,  "hysteria,"  is  usually  founded  on  these  fluctuating, 
sensory  signs.  Hoffman  and  Freund  found  sensory  disturbances  in 
from  70  to  90  per  cent,  of  their  cases.  Miiller  found  them  in  76  per 
cent.  Pains  are  not  frequent,  paresthesise,  on  the  other  hand,  are  very 
common.  Anesthetic  or  hyperesthetic  spots  are  frequent;  the  patients 
frequently  complain  of  dead  or  numb  fingers.  Tactile  insensibility  is 
not  infrequent,  occasional  tactile  agnosia  is  present.  The  sense  of 
localization  is  frequently  faulty,  and  bony  sensibility  is  also  often 
involved,  with  diminution  of  position  sense  and  disturbance  of  thermal 
sensibilities. 

Skin  Reflexes. — The  abdominal  and  epigastric  reflexes  are  almost 
invariably  modified  (80  per  cent.).  There  may  be  unilateral  diminu- 
tion or  loss,  or  more  often  bilateral  diminution  or  loss  (62  per  cent.). 
Thus  bilateral  loss  of  these  reflexes  constitute  a  very  important  differ- 
ential for  the  diagnosis  of  not  only  an  organic  nervous  disease,  but 
especially  for  multiple  sclerosis.  Careful  studies  by  Miiller  have  shewn 
that  when  correctly  tested  they  are  modified  in  only  about  5  per  cent, 
of  normal  individuals,  and  these  occurred  most  often  in  women  who 
had  had  children,  or  in  very  obese  subjects.  The  cause  is  to  be  sought 
in  an  interruption  of  the  sensory  tracts,  either  in  the  cord,  in  the  median 
lemniscus,  in  the  thalamic  end  stations,  or  in  the  post-Rolandic  sensory 
distributions.  The  multiplicity  of  the  sclerotic  patches  makes  it 
liable  that  this  reflex  path  will  be  implicated  somewhere  in  its  march  to 
the  cortex. 

The  cremasteric  reflex  is  also  often  involved  unilaterally,  or  bilater- 
ally, but  less  often.    Also  the  vomiting  reflex,  though  not  so  often. 

Babinski's  phenomenon  is  very  frequent;  Miiller  says,  almost  con- 
stant. Crossed  Babinski  may  even  be  observed.  Oppenheim  reflex  is 
very  often,  though  less  frequently,  found. 

Like  all  of  the  symptoms  of  multiple  sclerosis,  the  skin  reflexes 
are  subject  to  considerable  variations.  They  frequently  return  after 
showing  a  loss. 

Tendon  Reflexes. — In  concordance  with  the  spasticity  and  hyper- 
tonus  increased  tendon  reflexes  are  to  be  expected.  The  upper  ex- 
tremities show  increased  triceps-jerk  and  increased  radius-perioste.al 
reflexes.  The  Achilles-jerk  is  exaggerated,  unilaterally  or  bilaterally, 
as  also  the  knee-jerk,  and  usually  there  is  unilateral  or  bilateral  ankle- 
clonus.    Patellar  clonus  is  less  often  observed. 

Vasomotor  and  Trophic  Signs;  Other  Signs. — One  finds  a  number 
of  anomalies  of  this  group  in  a  large  collection  of  multiple  sclerosis 
cases,  but,  as  a  rule,  they  are  sparse  and  isolated.  Among  these  may 
be  mentioned  hyperidrosis,  angiospasms  (cyanosis),  vessel  palsies  with 
edema,  erythromelalgia-like  affections,  syringomyelic-like  trophic 
disturbances,  abnormal  blushing.  Hyperpyrexias  are  encountered 
with  apoplectiform  attacks.  In  the  ordinary  cases  the  patients  show 
marked  lability  of  the  body  temperature. 


MULTIPLE  SCLEROSIS  379 

Pilomotor  reflexes  are  frequently  disturbed;  thus  dermographia  is 
common,  and  may  show  unique  isolated  localizations. 

Bladder. — The  bladder  is  frequently  involved  (80  per  cent. — Oppen- 
heim;  75  per  cent. — Miiller),  often  transitorily,  and  also  it  may  be  an 
early  symptom.  The  patients  feel  a  sense  of  insufficiency,  and  have  to 
strain  to  pass  their  water.  Complete  paresis  with  incontinence  is  rare. 
There  is  a  great  variability,  with  many  ups  and  downs  in  the  bladder 
symptoms.     Polyuria  and  glycosuria  have  been  observed. 

Obstipation  is  frequent;   loss  of  control  less  so,  but  is  present. 

Menstruation,  childbirth,  parturition,  etc.,  are  not  markedly 
affected.  Genital  hyperesthesia  is  at  times  pathologically  increased 
and  occasionally  there  is  anesthesia  with  impotence. 

Lumbar  Puncture. — The  experience  thus  far  gained  shows  no  appre- 
ciable changes  in  pressure.  Cytologically,  occasionally  marked  slight 
lymphocytosis  has  been  observed,  but  the  majority  of  the  cases  have 
been  negative.  Serologically,  some  positive  Wassermann  reactions 
have  been  recorded.^ 

Mental  Symptoms. — These  are  not  constant  nor  prominent,  and 
are  for  the  most  part  absent,  yet  careful  analysis  shows  them  to  be 
much  more  frequent  than  is  usually  taught.  Mild  intellectual  reduc- 
tion in  the  form  of  a  dementia,  hallucinations  of  hearing,  of  sight,^ 
hallucinatory  states  with  mild  confusion,  passing  ideas  of  reference  and 
of  persecution,  difficulty  in  thinking,  spasmodic  intermittent  alterna- 
tions in  the  capacity  for  attention  and  concentration,  lapses  of  memory, 
etc.;  these  have  all  been  recorded.  The  latter  anomalies  are  not  in- 
frequent, and  often  are  colored  by  depression  (melancholic)  or  excited 
(manic)  states,  or  more  frequently  the  emotional  tone  is  one  of  indif- 
ference.    Pseud oparetic  states  may  be  expected  at  times. 

Involuntary  Laughing  and  Crying. — These  were  described  by  Cruveil- 
hier  in  his  classical  case,  and  correctly  appreciated  by  Bourneville. 
They  are  purely  neurological  signs,  for  neither  the  laughing  nor  the 
crying  are  necessarily  accompanied  by  their  usual  emotional  states, 
nor  do  they  necessarily  betoken  any  intellectual  reduction  (evidence 
against  the  James-Lange  emotional  hypothesis) .  They  are  very  often 
found,  forced  laughing  being  the  more  frequent  (40  per  cent. — Miiller). 
They  are  both  to  be  referred  to  implication  (in  part)  of  the  cortico- 
bulbar-cerebellar  reflex  paths.  An  analysis  of  all  of  the  variations  is 
not  possible  here. 

A-poplectic  and  Epileptiform  Attacks. — ^These  occur  but  not  as  fre- 
quently as  was  held  before  one  was  in  a  position  to  rule  out  anomalous 
paretic  attacks.  But  the  frequent  occurrence  (20  to  25  per  cent.) 
of  mild  attacks  with  transitory  disturbances  of  consciousness,  or 
attacks  of  giddiness  or  faintness,  with  unilateral  or  bilateral  pareses, 
or  sensory  anomalies  in  the  distal  extremities,  should  be  emphasized. 
Such  mild  attacks  resembling  arteriosclerotic  attacks,  often  mark  the 

1  Nonne,  Deut.  Zeit.  f.  N.,  1910,  1912.  ^  Nonne,  Mitt.  Hamb.  Staats  k.,  1910. 


380  LESIONS  OF  THE  SPINAL  CORD 

initial  stages,  or  accompany  the  sudden  progressions  which  are  char- 
acteristic of  this  disorder.  Epileptiform  attacks  are  much  less  often 
observed  and  usually  speak  against  a  multiple  sclerosis,  but  they  do 
occur,  and  very  frequently  show  as  Jacksonian  attacks  (Gussenbauer) . 

Characteristic  Forms. — As  already  noted,  the  classical  signs  of 
Charcot,  with  nystagmus,  intention  tremor  and  scanning  speech,  in 
reality  are  found  in  only  a  comparatively  small  proportion  of  the 
cases  in  the  earlier  stages  of  the  disease.  In  the  interest  of  early 
diagnosis,  this  must  be  born  in  mind,  especially,  as  true  nystagmus 
is  always  rare,  nystagmoid  movements  are  to  be  judged  with  care, 
and  the  tremor  and  scanning  speech  are  usually  later  symptoms. 
The  "non-typical"  cases  in  Charcot's  sense  (formes  frustes),  are  really 
more  frequently  met  with.  Any  attempt  to  state  which  special 
group  of  symptoms  occurs  with  more  or  less  frequency  is  apt  to  be 
misleading,  especially  in  view  of  the  great  variability  met  with  and 
the  inconstant  changes,  the  advance  of  certain  symptoms  and  the 
retrogression  of  others. 

For  purely  didactic  purposes,  one  can  divide  the  more  common 
symptom  pictures  as  follows : 

1.  Cases  that  begin  with  isolated  or  prominent  cerebral  symptoms. 
These  occur  less  frequently  than  the  spinal  cases,  perhaps  than  the 
bulbar,  but  hence  are  also  more  likely  to  be  overlooked.  Here  optic 
atrophies,  with  defects  of  vision,  ocular  palsies,  transitory  diplopias, 
crossed  eyes,  etc.,  muscular  weaknesses  are  prominent,  and  occasion- 
ally associated  with  giddiness,  nausea  and  headache. 

2.  Cases  with  isolated  or  prominent  bulbar  symptoms.  These 
begin  as  mild  or  severe  bulbar  palsies,  and  are  rare. 

3.  Cases  with  irregular  and  prominent  spinal  symptoms,  mostly 
involving  the  lower  extremities.  Here  the  patients  complain  of  the 
legs  becoming  tired  easily,  and  after  a  time  they  stumble,  or  trip 
easily.  They  then  become  somewhat  stiff  and  walking  upstairs 
becomes  increasingly  difficult.  Mild  bladder  disturbances  may  have 
preceded  or  accompany  the  weakness  and  stiffness  in  the  legs.  Par- 
esthesia are  also  frequent.  These  patients  soon  show  spasticity, 
increased  knee-jerks,  clonus  at  times,  Babinski's  sign.  The  abdominal 
and  epigastric  reflexes  are  diminished  or  absent  unilaterally  or 
bilaterally. 

This  is  apparently  the  most  frequent  mode  of  onset,  since  very  often 
the  patients  have  paid  no  attention  to  the  rapid  passing  of  transitory 
diplopias,  slight  speech  difficulties  or  weakness  of  the  bladder,  or  mild 
attacks  of  giddiness,  yet  sharp  questioning  usually  elicits  some  of 
these  other  signs  as  having  preceded  or  accompanied  these  spastic 
paraplegic  types.  Whereas  these  signs  usually  come  on  very  gradually, 
they  may  appear  to  have  had  a  sudden  onset,  as  after  a  long  walk,  or 
slight  accident,  or  following  childbirth,  or  other  striking  intercurrent 
event. 


MULTIPLE  SCLEROSIS  381 

4.  In  the  vast  majority  of  the  cases  there  is  a  gradual  onset  of  both 
spinal  and  cerebral  symptoms.  Headaches,  diplopia,  difficulty  in 
walking,  slight  changes  in  speech,  giddiness,  abnormal  muscular  tire, 
paresthesite,  transitory  bladder  weakness,  etc.  These  symptoms 
progress  and  then  recede — ^usually  attributed  to  the  skill  of  the  phy- 
sician or  thought  of  as  hysterical — and  then  reappear  in  the  same  or 
in  an  entirely  different  order. 

5.  A  small  number  of  cases  begin  with  a  mild  initial  apoplectiform 
attack,  and  then  either  progress,  or  stand  stationary  for  some  3'ears. 

Non-characteristic  Forms. — These  unusual  forms  may  be  summarized 
as  (a)  forms  which  run  a  more  distinctly  psychic  course,  with  the 
picture  of  slowly  advancing  dementia,  and  pseudoparetic  forms; 
(6)  forms  that  resemble  brain  tumor — hydrocephalus;  (c)  Jacksonian 
attacks  due  to  patches  in  the  motor  area;  {d)  hemiplegic  forms  due 
to  patches  in  the  cerebral  course  of  the  pyramidal  tracts;  (e)  bulbar 
paralysis  and  pseudobulbar  parah^sis  types;  (/)  sacral  forms  as  described 
by  Oppenheim,  resembling  tumor  of  the  cauda  equina;  {g)  forms  that 
give  an  acute  onset  resembling  pontine  encephalitis;  (A)  cerebellar 
types,  resembling  paralysis  agitans  (not  infrequently  called  early 
paralysis  agitans);  {i)  forms  that  give  a  picture  like  cerebrospinal 
syphilis;  (j)  progressive  muscular  atrophy  and  amyotrophic  lateral 
sclerosis-like  forms;  {h)  tabetic-like  forms;  (/)  transverse  myelitis  and 
combined  sclerosis  types,  and  finally  {m)  latent  and  recessive  forms 
which  are  differentiated  from  hysteria  neurological ly  only  after  many 
years  of  the  most  exact  scrutiny. 

Diagnosis. — Enough  has  been  said  to  indicate  how  a  multiple  sclerosis 
may  appear  under  the  guise  of  a  number  of  organic  diseases  of  the  brain 
and  spinal  cord.  The  most  important  features  in  the  diagnosis  concern 
the  age  of  the  patients,  young  individuals,  the  usual  lack  of  heredity, 
the  failure  of  usual  external  causes  to  account  for  the  symptoms, 
absence  of  Wassermann  reactions  and  of  spinal  fluid  findings.  Of  the 
more  important  objective  findings  for  the  early  diagnosis  one  counts 
on  the  early  feeling  of  motor  weakness,  feelings  of  giddiness,  the  onset 
of  spasticity  with  increased  reflexes,  clonus  and  Babinski,  the  presence 
of  the  fundus  changes,  the  loss  of  the  abdominal  reflexes,  fine  ataxic 
movements  of  the  legs  in  the  knee-heel  test,  and  of  the  arms  in  the 
finger-finger  and  finger-nose  tests. 

In  the  later  stages  the  full  pictures  as  already  outlined  appear 
and  make  a  diagnosis  certain.  One  feature  of  perhaps  the  most 
striking  importance  is  the  peculiar  advancing  and  receding  course, 
the  remissions  during  which  the  entire  picture  seems  to  fade  away 
and  which  permit  a  patient  condemned  to  a  chronic  organic  nervous 
lesion  to  turn  up  in  the  physician's  consulting-room  apparently  well. 

It  is  for  this  latter  reason  perhaps  more  than  any  other  that  the 
false  diagnosis,  hysteria,  is  so  frequently  made  in  these  cases.  Then 
there  are  in  addition  the  almost  daily  fluctuations  in  the  sensory 
sphere  which  always  suggest  hysteria,  and  which  only  a  rigid  analysis 


382  LESIONS  OF  THE  SPINAL  CORD 

will  exclude.  Furthermore  it  must  not  be  forgotten  that  not  only  does 
one  experience  the  changeability  in  symptomatology  suggestive  of 
hysteria,  but  a  helpful  optimistic  psychotherapy  can  most  markedly 
alter  the  symptoms  as  well,  causing  some  of  them  to  entirely  disappear. 
The  rise  in  the  level  of  nervous  energy  by  such  psychic  means  seems  to 
enable  the  patient  to  force  a  better  control  of  the  disturbances.  In  the 
failure  of  a  typical  hysterical  character — the  hysterical  constitution — 
this  disorder  should  not  be  diagnosed.  Finally  one  has  always  to 
reckon  with  a  symptomatic  hysterical  reduction  in  the  patient's 
resistance  due  to  the  presence  of  an  organic  lesion. 

Further,  diffuse  sclerosis,  general  paresis,  brain  tumor,  chronic 
hydrocephalus,  arteriosclerosis,  cerebral  syphilis,  infantile  palsies, 
encephalitis  of  a  diffuse  disseminated  type,  bulbar  palsies,  chronic 
leptomeningitis,  infantile  ataxias,  Friedreich's  ataxia  in  particular, 
disseminated  myelitis,  meningomyelitis,  Erb's  spastic  palsy,  heredi- 
tary spastic  palsies,  syringomyelia,  tabes,  spinal  cord  tumor,  paralysis 
agitans,  chronic  zinc  poisoning,  chronic  manganese  poisoning,  chronic 
mercurial  poisoning  and  spinal  cord  edemas  all  come  in  review  in  the 
differential  diagnosis  of  multiple  sclerosis. 

Pathology  and  Pathogenesis. — On  autopsy  in  the  cases  of  true  multiple 
sclerosis  one  finds,  as  a  rule,  a  normal  dura,  the  brain  itself  usually 
shows  irregular  atrophies  with  thinning  of  the  cortex  and  some  internal 
hydrocephalus.  At  times  one  can  observe  on  the  surface  the  irregular 
patch-like  areas  of  atrophy.  These  usually  show  much  more  frequently 
on  the  surface  of  the  pons,  medulla  and  cord,  as  Carswell  and  Cruveil- 
hier  showed  early.  On  section  of  the  brain  one  finds  few  (five  or  six) 
or  many  (several  hundred)  irregular  sclerotic  patches,  which  are 
usually  isolated  one  from  another. 

Microscopic  examination  invariably  reveals  many  not  seen  by  the 
naked  eye.  They  vary  in  size  from  that  of  a  pin-head  to  5  to  6  cm., 
which  larger  patches  are  usually  made  up  of  several  smaller  ones.  The 
general  color  tone  is  gray.  The  reddish  patches  usually  belong  to 
the  secondary  encephalomyelitides.  The  plaques  either  rise  slightly 
from  the  surface  or  are  level  or  show  a  slight  depression;  the  tissues 
about  are  slightly  edematous.  At  times,  particularly  in  the  optic 
nerves,  there  is  distinct  shrinkage  in  the  tissues.  The  consistency 
is  usually  tough,  or  almost  leathery.  The  soft  plaques  are  apt 
to  resemble  disseminated  myelitis,  encephalomyelitis,  etc.  The 
patches  are  very  sharply  differentiated  from  the  surrounding  tissues. 
As  to  localization,  they  may  be  anywhere;  in  the  brain,  cord,  medulla, 
pons,  cerebellum,  the  roots  of  the  cranial  or  spinal  nerves,  within  the 
optic  nerve  itself.  As  a  rule  they  are  found  in  both  brain  and  spinal 
cord,  and  seem  to  have  a  special  fondness  for  places  particularly  rich 
in  neuroglia,  and  there  is  a  certain  grade  of  asymmetrical  symmetry 
in  their  localization. 

In  the  cord,  the  median  line,  and  the  pyramidal  tract  region  are 
favorite  localizations,  and  the  white  matter  more  than  the  gray  above 


MULTIPLE  SCLEROSIS  383 

all.  Central  gliosis  seems  rare.  In  the  medulla  the  olivary  region, 
the  floor  of  the  fourth  ventricle,  and  the  posterior  aspects  are  favorite 
sites,  while  in  the  cerebellum  the  dentate  nucleus  is  usually  most 
often  implicated. 

Histologically  the  patches  are  made  up  of  masses  of  glia  fibers. 
There  is  no  areolar  composition  and  the  glia  nuclei  do  not  appear 
prominent.  Small  holes  may  be  found  within  the  plaques,  around 
which  the  glia  fibers  cluster  as  about  a  bloodvessel.  Bielschowsky 
preparations  show  the  partially  intact  nerve  axones  passing  through 
the  glia  masses  in  much  reduced  numbers;  the  medullary  sheaths  are 
partly  modified  in  their  qualities.  Charcot  first  called  attention  to  the 
persisting  axis  cylinders.  The  immense  mass  of  glia  fibrils  is  an 
entirely  new  growth,  apparently  not  to  take  the  place  of  destroyed 
nervous  tissue,  but  a  pure  addition  product  in  which  one  finds  the 
almost  normal  elements  still  present,  for  a  time  at  least.  Secondary 
degeneration  outside  of  the  plaques  is  not  present. 

What  relation  the  disease  has  to  the  vessels  is  still  far  from  clear, 
but  it  seems  certain  that  primary  disease  of  the  vessels  is  not  a  part  of 
true  multiple  sclerosis. 

The  differentiation  from  disseminated  myelitis,  encephalomyelitis, 
and  other  secondary  affections  which  may  give  rise  to  partial  multiple 
sclerosis  pictures  must  be  sought  in  special  works  on  pathology. 

Prognosis. — In  general  the  disorder  is  progressive,  yet  there  are 
many  stationary  cases,  and  some  few  that  apparently  recover;  these 
are  possibly  mistakes  in  diagnosis,  but  such  mistakes,  in  view  of  the 
great  number  of  similar  affections,  are  almost  impossible  to  avoid. 
Many  patients  extremely  ill  and  bed-ridden,  following  one  of  the  acute 
advances  of  the  disease,  recover  almost  completely;  but  usually  there  is 
another  acute  advance,  and  then  others.  The  time  between  advances 
or  the  length  of  remissions  cannot  be  stated;  they  have  varied  from 
six  months  to  ten  years;  some  very  rare  observations  show  a  period  of 
twenty  years. 

A  few  patients  die  rapidly  of  the  disorder  (six  weeks  to  six  months) 
— acute  multiple  sclerosis  of  Marburg,  Frankl-Hochwart  and  others — 
but  the  majority  live  for  many  years,  and  die  usually  of  intercurrent 
disorders,  pyelitis,  tuberculosis,  pneumonia,  etc.  Miiller's  average 
was  four  years;  Charcot's,  six  to  ten  years. 

Therapy. — Specific  therapy  is  not  yet  known.  Prophylaxis  also 
seems  difficult  to  grasp  since  no  definite  exciting  cause  is  known. 
Relative  prophylaxis  in  the  shape  of  advising  against  marriage  for 
young  affected  individuals,  or  against  childbearing  in  the  married  is 
necessary.  Childbirth  would  seem  to  be  an  exciting  cause  for  an 
active  exacerbation. 

Rest  in  bed  is  more  than  desirable  in  acute  stages,  it  is  imperative. 
The  rest  must  be  absolute,  and  should  include  eye  rest.  Active 
remedial  treatment — massage,  hydrotherapy,  electrotherapy — are  all 
disadvantageous.     Warm  baths   for   short   periods   are   not   contra- 


384  LESIONS  OF  THE  SPINAL  CORD 

indicated.  Definite  motor  quiet  and  sensory  quiet  as  well  is  desirable. 
Counter-irritation  in  those  cases  which  show  sharp  myelitic  symptoms 
is  to  be  tried. 

Pharmacotherapy  has  not  yet  devised  any  useful  remedies.  Arsenic 
is  used  on  empirical  grounds  and  in  combination  with  quinin,  ergot, 
strychnin  and  iron  does  some  service. 

Mild  work  in  the  open  air,  gardening,  etc.,  is  advisable.  Psycho- 
therapy is  never  to  be  forgotten,  and  a  healthy  optimism  helps  these 
patients  enormously. 


CHAPTER  VIII. 
DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN. 

Disease  of  the  brain  stem  offers  special  problems  of  diagnosis  of 
extreme  difficulty,  both  with  reference  to  the  motor  and  sensory 
disturbances.  Attention  will  first  be  directed  to  the  latter.  (See  Plates 
X  and  XI.) 

So  long  as  the  sensory  paths  were  in  their  spinal  route  they  were 
capable  of  a  certain  amount  of  isolation  either  as  they  entered  the  cord 
and  made  their  first  synapses,  or  as  they  continued  up  the  cord  in 
primary  or  secondary  paths.  But  as  these  paths  converge  to  enter 
the  brain  stem  they  become  closer  anatomically,  disease  processes  are 
apt  to  overrun  many  paths,  and  thus  th'e  analysis  becomes  increasingly 
difficult  up  to  the  entering  of  these  paths  into  the  optic  thalamus. 

Head  and  Holmes  have  made  the  most  searching  analyses  and  their 
researches  tend  to  show  that  the  impulses  underlying  sensations  of 
pain,  heat,  and  cold  seem  alone  to  run  unaltered,  either  directly  or  by 
intercalated  fibers  associated  with  the  ganglion  cells  of  the  formatio 
reticularis,  between  the  upper  end  of  the  spinal  cord  and  the  optic 
thalamus.  Here  are  received  the  regrouped  secondary  impulses 
from  the  face  which  cross  then  join  the  specific  paths  for  pain,  for 
heat  or  for  cold.  These  paths  are  so  situated  that  they  can  be  inter- 
rupted without  disturbance  of  any  other  form  of  sensation  of  the  body, 
and  the   analgesia  and  thermo-anesthesia  so  produced  resemble  in 

General  Legeistd  Abbreviations  of  Pontine,  Peduncular,  and  Midbrain 

Syndromes. 

In  all  of  these  hemiplegia  is  indicated  by  oblique  lines;  hemianesthesise  by  dots,  and 
alternate  paralyses  by  gray  network.  The  lesions  involve  the  cerebral  peduncles  and 
the  pons  at  different  levels. 

■  Abbreviations.  Aq.,  aqueduct  of  Sylvius;  BrQp,  peduncle  of  posterior  corpus 
quadrigeminum ;  •  Crst,  corpus  restiforme  and  inferior  cerebellar  peduncle ;  Fee,  central 
tegmental  tract;  Fes,  internal  semicircular  fibers  of  the  cerebellum;  Flp,  posterior 
longitudinal  fasciculus;  FPoa,  FPop,  anterior  and  posterior  pontine  fibers;  HC,  cere- 
bellar hemisphere;  Lc,  locus  ceruleus;  LN,  locus  niger;  Lig,  lingula  of  superior  vermis 
of  cerebellum;  ND,  Deiters'  nucleus;  Np,  nuclei  of  pons;  NR,  red  nucleus;  NRl, 
nucleus  of  lateral  lemniscus;  Nrt,  reticular  nucleus  of  the  tegmentum;  NIII,  nuclei 
of  third  nerve;  NmV,  motor  fifth  nucleus;  NsV,  sensory  fifth  nucleus ;  NVI,  nucleus  of 
sixth  nerve;  NVI  I,  nucleus  of  the  facial  nerve;  NVIII,  anterior  nucleus  of  the  acoustic 
(cochlear);  Oc,  cerebellar  olive;  Os,  superior  or  pontine  olive;  P,  lower  stage  of  cere- 
bral peduncle;  Pcm,  middle  cerebellar  peduncle;  Pes,  superior  cerebellar  peduncle; 
■Py.  pyramidal  tract  in  its  pedunculo-pontine  region;  Qa,  corpus  quadrigeminum  anterior; 
r,  raphe;  Rm,  median  lemniscus;  Rl,  lateral  lemniscus;  SAq,  gray  substance  of  the 
aqueduct  of  Sylvius;  (Sgr 72,  substance  of  Rolando;  Tpo,  tenia  pontis;  7"?-,  trapezoid  body; 
Vi,  fourth  ventricle;  Vs,  superior  vermis  of  the  cerebellum;  VV,  valve  of  Vieussens; 
///,  root  fibers  of  the  third  nerve;  V,  trigeminus;  Vc,  descending  motor  root  of  the 
fifth;  Frfs,  descending  sensory  root  of  the  fifth ;  F"/,  root  fibers  of  the  sixth;  VII,rVII, 
root  fibers  of  the  facial;  VI Ig,  knee  of  the  facial;  VIIIv,  vestibular. 
25 


386     DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN 

quality  the  loss  of  sensation  to  pain,  heat,  and  cold  caused  by  a  lesion 
in  the  spinal  cord.    (See  Plates  X  and  XL) 

Thus  when  a  lesion  of  the  bulb  interferes  with  sensation  of  pain,  not 
only  may  the  skin  be  insensitive  to  prick,  but  the  readings  of  the 


Fig.  195. — Posterior  peduncular  syndrome.  Contralateral  Benedict's  syndrome. 
Hemianesthesia  with  choreo-athetoid  movements  and  alternate  paralysis  of  the  third 
(777)  nerve  by  reason  of  lesion  of  the  right  pontine  tegmentum. 

On  the  left  side,  contralateral  hemianesthesia  of  the  extremities  and  of  the  face  from 
lesion  of  the  secondary  crossed  sensory  pathways  (Rm  and  SR).  Choreo-athetoid  move- 
ments and  at  times  tremors  from  lesion  of  the  red  nucleus  and  of  the  superior  cerebellar 
peduncle  (Pes)  below  the  decussation. 

On  the  right  side  paralysis  of  the  third  nerve  (III),  with  ptosis,  external  strabismus 
(non-resistance  to  external  rectus),  with  or  without  mydriasis  and  pupils  immobile  to 
light  and  convergence,  depending  on  the  extent  of  the  lesion  of  the  root  fibers  of  the 
oculomotorius  (777).     (Dejerine.)     (See  Chart,  No.  6,  p.  390.) 


pressure  algometer  may  be  raised  on  the  analgesic  side.  In  the  same 
way  the  affected  area  of  the  body  may  be  insensitive  to  all  degrees  of 
heat,  and  to  all  stimuli  capable  of  evoking  normally  a  sensation  of 


PONTINE  SYNDROMES  387 

cold.  Here,  however,  in  the  bulb,  in  distinction  to  lesions  of  the  cord, 
the  grosser  form  of  pain  and  discomfort  may  traverse  other  paths  if 
the  usual  ones  are  closed,  whereas  in  the  cord  all  painful  impulses  are 
blocked  by  a  unifocal  lesion. 

In  the  bulb,  moreover,  all  three  forms  of  sensibility  may  be  affected 
together  or  any  one  may  escape  or  be  alone  involved. 

These  impulses  of  pain,  heat,  and  cold  all  run  up  in  the  neighborhood 
of  the  fifth  nerve  nucleus,  and  in  cases  of  occlusion  of  the  posterior 
inferior  cerebellar  artery  the  paths  are  usually  implicated.  This  same 
accident  may  occasion  a  dissociation  of  the  impulses  underlying  the 
appreciation  of  posture  and  passive  movement  from  those  concerned 
with  spatial  discrimination. 

A  summary  of  the  findings  which  may  occur  in  the  lesions  which 
cut  off  the  sensory  pathways  between  the  nuclei  of  the  posterior 
columns  and  the  optic  thalamus  has  been  stated  by  Head  and  Holmes 
as  follows: 

1.  The  impulses  for  pain,  heat,  and  cold  continue  to  run  up  in 
separate  secondary  paths  on  the  opposite  side  of  the  nervous  system 
to  that  by  which  they  entered.  They  receive  accessions  from  the 
regrouped  afferent  impulses  from  the  nerves  of  the  head  and  upper 
part  of  the  neck. 

Although  these  paths  are  frequently  affected  together  they  are 
independent  of  one  another,  and  any  of  the  three  qualities  of  sensation 
may  be  dissociated  from  the  others  by  disease. 

2.  Lesions  of  the  spinal  cord  tend  to  diminish  simultaneously  all 
forms  of  painful  sensibility,  but  with  disease  of  the  brain  stem  the 
gross  forms  of  pain  and  discomfort  may  pass  to  consciousness,  although 
the  skin  is  analgesic.  This  applies  not  only  to  painful  pressure,  but 
to  the  discomfort  produced  by  excessive  heat. 

3.  The  impulses  concerned  with  postural  recognition  part  company 
with  those  for  spacial  discrimination  at  the  posterior  column  nuclei. 
Up  to  this  point  they  have  travelled  together  in  the  same  column  of  the 
spinal  cord,  but  as  soon  as  they  reach  their  first  synaptic  junction  they 
separate.  Above  the  point  where  they  enter  secondary  paths,  the 
power  of  recognizing  posture  and  passive  movements  can  be  affected 
independently  of  the  discrimination  of  two  points  and  the  appreciation 
of  size,  shape  and  form  in  three  dimensions. 

4.  It  would  seem  as  if  those  elements  which  underlie  the  power 
of  localizing  the  spot  touched  or  pricked  become  separated  off  from  their 
associated  tactile  impulses  before  they  have  actually  come  to  an  end 
in  the  optic  thalamus.  The  long  connection  of  localization  with  the 
integrity  of  tactile  sensibility  is  here  broken  for  the  first  time. 

All  these  changes  are  preparatory  to  the  great  regrouping  which 
takes  place  in  the  optic  thalamus. 

The  analysis  of  the  motor  pathways  is  equally  involved.  For 
economy  in  space  and  for  convenience  of  reference  the  various 
syndromes  are  here  tabulated.^     (See  Plate  I.) 

1  Kindness  of  Dr.  F.  Tilney  in  collaboration. 


388     DISEASES  OF  THE  PONS,  BRAIN  STEM,   AND  MIDBRAIN 


Fig.  196. — ^Anterior  peduncular  syndrome.  Weber's  syndrome.  Superior  alternate 
hemiplegia.     Produced  by  cerebral  peduncle  and  ///  root  fiber  lesion. 

On  the  right  side,  contralateral  hemiplegia  of  the  trunk  and  extremities  from  lesion 
of  the  corticospinal  pyramidal  fibers.  Facial  hemiplegia  inferior  branches,  hemiparesis 
of  the  tongue  from  lesion  of  the  corticonuclear -fibers  of  the  hypoglossus.  In  (a)  the 
lesion  involves  only  a  portion  of  the  corticospinal  and  corticonuclear  fibers.  In  (b)  the 
lesion  includes  all  of  the  pyramidal  corticospinal  and  corticonuclear  fibers,  even  those 
which  in  this  region  form  the  superficial  and  deep  pes  lemniscus  fibers.     (See  Plate  I.) 

In  addition  to  the  symptoms  common  to  (a)  and  (6)  indicated  in  the  illustrations  (b) 
also  shows  a  conjugate  deviation  of  the  head  and  the  eyes  from  a  lesion  of  the  cortical 
oculorotary  fibers  destined  to  go  to  the  nuclei  of  the  oculomotorius  and  abducens 
(III-VI);  difficulties  in  mastication  from  lesions  of  the  motor  corticotrigeminus  fibers; 
difficulties  in  swallowing,  of  phonation,  and  of  articulation  from  lesions  of  the  cortico- 
nuclear fibers  of  the  spinal  vagus. 

As  a  result  of  the  predominance  of  the  antagonists  of  the  sound  side  (left)  in  (a)  and 
(6),  there  can  be  observed  a  deviation  of  the  mouth  (facial),  and  of  the  jaw  (masticators) 
toward  the  left,  a  deviation  of  the  tongue  to  the  paralyzed  side  (action  of  sound  genio- 
glossus)  and  in  (b)  further  a  conjugate  deviation  of  the  head  and  of  the  eyes  toward  the 
left  (action  of  the  external  branch  of  the  spinal  accessory  and  of  the  levorotory  ocular 
fibers)  and  a  deviation  of  uvula  toward  the  left  (spinal  vagus) . 

On  the  left  side  there  is  a  direct  paralysis  of  the  third  (III)  nerve  with  ptosis,  divergent 
strabismus,  with  or  without  mydriasis  and  pupils  which  do  not  react  either  to  light  or 
convergence,  according  to  the  grade  of  destruction  of  the  root  fibers  of  the  oculomotorius 
(777).     (Dejerine.)     (See  Chart,  No.  5,  p.  390.) 


PONTINE  SYNDROMES 


389 


ff-^i7/eC 


Fig.  197. — Anterior  cephalic  pontine  syndrome.  Hemiplegia  of  cerebral  type  due  to 
thrombosis  of  the  upper  part  of  the  basilar  trunk.  The  lesion  is  unilateral,  occupying  the 
cephalad  part  of  the  pons,  or  the  anterior  portion  of  the  right  pons  destroying  there  the 
corticospinal  pyramidal  fibers,  the  corticonuclear  fibers  of  the  facial,  the  masticators, 
and  the  hypoglossal.  It  does  not  involve  the  tegmentum  nor  the  root  fibers  of  the 
cranial  nerves. 

On  the  left  side  there  is  a  crossed  hemiplegia  of  the  trunk  and  extremities  with  con- 
tractures and  exaggeration  of  the  reflexes.  Left  inferior  facial  hemiplegia,  mild  superior 
hemifacial  paresis,  slight  widening  of  the  palpebral  fissure,  slight  drooping  of  the  external 
border  of  the  left  eyebrow,  slight  impairment  of  independent  closure  of  the  eyes;  hemi- 
paresis  of  the  masticators,  especially  of  the  internal  pterygoid.  Hemiparesis  of  the 
tongue.    Integrity  of  electrical  reactions. 

By  reason  of  the  predominance  of  the  muscles  on  the  sound  side  the  lips  and  jaw  are 
drawn  to  the  right  and  on  protrusion  of  the  tongue  it  points  to  the  paralyzed  side  due  to 
action  of  the  right  genioglossus.     (Dejerine.) 


390     DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN 


o3-g  O 
■«)«■?, 


in 


a  o 

'as 
sa 


'—'      '^- 


c« 


CO  03- 
a  <3 

o 


a-^g- 

ab"'" 
^a„ 

_^   CI   ^ 

s .co'^roS  S„^ 

»   O)   C3     .   JJ-y   S 

§-§'g.sfasiS 

PQ  H 


'-  2.S    . 

3m  a  s 


a— 

6^ 


a^j 

a>  a 

?'s 

a_ 

"3 -2 
a^ 
o 


M  i  S  S 

CD    g   C    (U 

oT3  CI  S 
ffi  CI  a>.5 

a  "j3 

o^     -  O 
._^   03  S(^     ■ 

laa'ol 


1 

a 
1 

Contralateral       hemiplegia; 
convulsions      (Jacksonian) 
common;  aphasia  frequent, 
intelligence  often  disturbed; 
may  be  monoplegia. 

Contralateral  hemiplegia; 
Jacksonian  convulsions 
rare;  monoplegia  rare;  intel- 
ligence seldom  disturbed. 

Contralateral  hemiplegia; 
convulsions,  aphasia,  and 
mental  disturbance  atasent. 

Contralateral  hemiplegia; 
hemianesthesia ;  hemia- 
taxia;  hemiathetosis  and 
sometimes  hemianopsia; 
pain  in  affected  parts. 

Contralateral  hemiplegia  in- 
volving face,  tongue,  arm, 
and  leg;  ipsilateral  ophthal- 
moplegia. 

Contralateral  hemichoreo- 
athetosis;  hemitremor  and 
hemianesthesia;  ipsilateral 
external    ophthalmoplegia. 

Contralateral  hemiplegia; 
hemipropoplegia;  hemi- 
paresis;  masticatory  hemi- 
paresis;  lingualis. 

Complete  hemiplegia  of  one 
side;  partial  hemiplegia  of 
other  side;  paralysis  of 
both  sides  of  face;  paraly- 
sis of  both  sides  of  tongue 
with  dysarthria. 

Contralateral  hemiplegia; 
contralateral  paralysis  of 
VII  and  XII  nerves;  con- 
tralateral fixed  conjugate 
deviation  in  extreme  lateral 
position ;  cannot  move  eyes 
out  of  this  position;  looks 
toward  paralyzed  limbs. 

S  3jS':3  o3 
S   £   g'^   S) 

^  »  S  a  e 
_--Q  CJ  a?-s 

§5"c  § 

^   -  .-  "^  S 
°  I.  2  o 
,c  o  +i  M  o 

S   g'M.S'-i? 
■^  3  «  CI  „ 

a    §  a  3  s-^ 

.2  cu"  c      cs-g 


M  O 


.2  CD-S        g-S  "^ 
a  d       CD       o  °3 

o-s  §"-s  CO- a  § 


S° 


a  i  s 

S  ff  '^ 
a  a  o 
K  2  m 

"^■^ 
o  B  c   . 

g  0)  o3  a) 

a  ill 


a--^ 


^5  = 
"1% 


2  P  >> 


3  2 


^aS 

rft    Ci     o 


tr  CO  T^ 


OJ   3  t<        o3M  gi-H 

«2S-Sfe:Sa§ 


cB-rt     o3-q 


S  03  (N   3i-H 


-     ^~      o^ 


J>        i  l.'3>5 


^8S      2  9^2° 


a.> 


■-^  =3  S 


O   03--S   U 

O  o  go 

.2  >>    . 

;2^+3  a;!;'- 


-g  03  c 
c^';3 

•aaJ 


.y-gcca.2      3_£ 
S  S  a|=|  a-S  §     « 

<D  a  (D     g  «•"  ® 
;:;^  a  03  bjo;:^  a  h  0  fi 


*3  ™ 

«  !3  & 
2  a  CD  ^ 

■2gt:l 

c;^  03 


a^S-^ 


a  (D 


0J,2  m        3 
■S  3  >        g 


.^      o  g 
'i^.tH  2  " 


S-S323c: 

O    S    >)J3   CD 

;:5- a o  ao  n 


°  s 
a'3 

S  03 

3  F-i 

a  c 

ID  o 

O 


o  2 
a  a 


r!   "^ 


a  3_^ 
aj 

03 


^-ai  3-et 

a  S'3  a  a  c 
O        O 


PONTINE  SYNDROMES 


391 


H.  (fillet, 
FPoa. 


Fig.  198. — Anterior  and  posterior  internal  pontine  syndrome.  Thrombosis  of  the 
middle  portion  of  the  basilar  artery  involving  the  right  anterior,  mesial  basotegmental 
portion.  The  lesion  not  going  as  far  as  the  motor  and  sensory  nuclei  of  the  trigeminus 
and  avoiding  the  posterior  longitudinal  fasciculus.  There  is  contralateral  hemiplegia 
on  the  left  side,  of  the  cerebral  type,  of  the  trunk  and  extremities  and  of  the  inferior 
facial  by  reason  of  the  lesion  of  the  corticospinal  pyramidal  and  facial  fibers  (Py). 
Mild  hemiparesis  of  the  upper  facial,  hemilingual  paresis,  and  hemiparesis  of  the  masti- 
cators (deviation  of  the  chin  to  the  sound  side  by  implication  of  the  aberrant  pyramidal 
corticolingual,  and  corticotrigeminal  (motor)  fibers  which  travel  in  the  median  lemniscus 
(i2m)  and  the  tegmentum  (Sr)).    (See  Plate  I.) 

There  is  contralateral  hemianesthesia  for  all  kinds  of  sensibility  but  above  all  of  the 
sense  of  position  by  lesion  of  the  median  lemniscus  (Rm)  and  of  the  reticular  formation 
of  the  tegmentum  {SR).  There  is  no  alternate-paralysis,  the  lesion  not  involving  any 
of  the  sensory  or  motor  cranial  nerve  nuclei.    (Dejerine.)     (See  No.  11  in  Chart,  p.  .392.) 


392     DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN 


'5b"o 

O  m  O 
-g  g  CD  g  5 


O  M 


C-2 
1)  <u 


—  c   ■ 

W-.  ^j  I— I 


*a 


S3 

bO 

'S 

-S 

a 

d 

0) 

fl 

p^ 

O 

a 

bll 

^■a 

a 

-C 

P 

a 

S  E 


-2  gfe 

T3   5?   CD 

"See 


03  a>  C  jH    ■ 

0,03  M-^ 
g  ^  '^■S 


■IT  L,  '^ 

2'a- 
go 

J  5PS 


i5r3. 


3  jjri  ft 


s  dp  ■ 


O    m    "I'd 
■t^^   C   aJ   |H 


'Si"  03  §  =3  ft 
ftg  -^  M|1> 
C  ft  d  03-p  3 


I'ii 


13  S-i  ii 
03^   S   h  O 


^        &-iJ.M 


.>>-^  3  2-^' 


c3  „r      -^  — c 


03, "^la       -^P        SS?» 
—  ^         "   ?n  03-5.2  OJ   S"^ 

o  o  o  g"*-  (u  5  CD- 


>>+J  c3' 

03    « 

a.S' 
"3 


— •       -^  C  o3 

Q  (!3  "tJ  V  ft 

H   t^  (D  o3  j:3  "^ 

?      i^  ^      O  t4 


OJ.S  03  53      JS"" 


373   3  S  H 


£3   03_ca   o 
3   S        03 

3      •-  "^la 


a>-      03^- 


S  ft  °  -^,  g  m 

u  o3  m  S  £^'~' 
3  a3iJj=-C  c3  ^ 


J3OT      ^ro'-3.-Ho3 

S  os-Sn-^agg  ft 

'S  m  <u  S 


OJ  o3 
a)_a+J 

03  a_ 

*■  (D  C3 
«-3  C 

O  ^^ 

a) . 


S  >io3  to 
n,  ra  aj  jj 

o3   3   5; 


_   =3 

+^  u  03  a 

03   o  fi„ 


oyC^^Mr.'^^j         -^03 

■-d"§-s  <u  fc  °  g-3  S- 

Ca    S;Jj+J    03    t1    03*^    rn 


^  g  a  3    g  ft+j 


,2  "-rt-^^-S" 


__H   M  o3   M  5R^  ' 


tri  ca   .  ^' 

ft"S-S 


5  o  C  03  »  o  +J  ■ 


-2  03 
-  3  M-- 
n  3  03 


g.^-H-ca 


ag  "S 


a  3  m  .S 

o.2':3'g 


.B-a- 


3  a  o 


o  03  03-; 


■a° 


0)  "^  a 
be  ■"  S? 

o3  a)  " 

Sea 


a    OJ    GQ 

P-Q'k  od 
tl  3  P3 


3  a> 


a^ 


■so     ^3 


o*J  3 
ca   3  o 

h:3  a 


^^1 


O      O      tH    "^ 

o  C 

°i-r 

rs; 
yric 
ear 
erve 

_> 
N 

.-XI  o's  ^ 

> 

i3«13  2t:j 

•r 

■3.S-g.§M 


>.a'g'~'""_o 


jro  3      y3 

...    <D     3     tH 


o  s'o'S'g  g    • 
3.2  fe  >>o  S 

;5 a+j  o  ao  3 


3  3  Ji  "^ 


3^03 


PhIO  03^ 

•-1 --Si 


fen   03, 


N  «  s 


<N  3  ^ 

■So.        "I*^ 

a>|^g^°  . 

^i^  S  2^M:2  2 


iJ  03 


,S  03  ^' 


Ph   " 


PONTINE  SYNDROMES 


393 


Fig.  199. — Pontine  middle  syndrome.  Lesion  of  the  lateral  part  of  the  left  pontine 
tegmentum,  involving  the  nuclei  of  the  trigeminus,  the  crossed  secondary  sensory 
paths  of  the  tegmentum,  partially  involving  the  superior  cerebellar  peduncle  (Pes) 
and  the  median  lemniscus  (Rm),  and  not  involving  the  anterior  portion  of  the  pons. 

On  the  right  there  is  hemianesthesia  of  the  extremities  of  the  syringomyelic  type, 
above  all  for  pain  and  temperature  sense. 

On  the  left  there  is  paralysis  of  the  muscles  of  mastication  (pterygoid,  masseter, 
temporal)  by  lesion  of  the  motor  nucleus  of  the  trigeminus.  There  is  slight  anesthesia 
in  the  trigeminus  region  (sensory  nucleus  TO  and  choreo-athetoid  movements  of  the 
extremities  from  involvement  of  the  superior  cerebellar  peduncle.  (Dejerine.)  (See 
Chart,  No.  13,  p.  392.) 


394     DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN 


M    rt    fl 

'-  £  >> 

■-M  fi  '^  £: 


bO^  M 


«.: 


c  g  S.B-3   . 


„j  C  C3  cS  OJ 


-S^ 


oi— .  S  1 


o 


«£-S 


55  i^ 

o3  a  ho 


3  g  ^"3 .5  ° 

>>^  aboS  2 
g  m  S  ag  c 

o 


^1  §l2 
I--—  03  a  ho 


'x  P 


as 


-G  eS. 


k1  M  »  t,  c3  2  ■ 

O  ' 


o-2'o"*! 

fin 


3  "3  S  =3  Ji  'f£'o 


Hi   0-r<,£5   >i 


0  o  rl.g'S-§-^'M'5 

*        fU  c!  ™ 


:  o3jq 

03 


,s  s  S  ^ 

^   aJ-3  & 


-^  s  o  S 
t<  2  1^ 

t.    CD    fl  -M 


■3  ca     — 
a  M    „ 


v-<*:?  c3  tH  £ 
o3  cl  t-  <p  S-- 


3^'"^-3  ra  "  - 


i  0)  c  »  S 
3:3  o-S  "3 


ca  o3 

!  ^  "?^  S  ^  ^ 


S  §•«    ^  a 
M^-  .  §  _•- 

o  ars  ^^■5  "J^ 
R|g.S  a|a 


C!  03  2 
•11 


a' 


-2      ^  S 

bD  =3rt  S 


-ca-S^-S 


-".2  03 

0)  2 

,-H  CI  2 

OJ  03  • - 


M  O-S  03 
OJ  bt  03  „ 
-:;  c3  tH  R 

o  p  £3  ^ 

a  ^-.2  ^ 

.^'bllO'm  I 

IX  a  K  -M  I 


(S    O    r^ 

■2-^    CO 

aS- 


^T3 
S  S  ro 

'bo's 
0) 


-s.s    ^ 


^  S-a 


«lo3-S  >>§ 
O   ?<   C   w   o 

-i-e  "  is  a-S 

bO  >)  03^ 

—  r:i  a 
a  b  '"  " 


ai  bO  a      03 
g,^  bO?  03 


O  o3 

n  03 
o.S 
^& 
H 


O'CD 


e.  o  ; 
^ai 


aj'fl 


3 


^         a) 
2   >>« 

a  sc^ 

aj  C  O 


05  srS  3 

^-'S  a_» 


-l  2  bO 


3--2 


Q        = 


i^M-S-S" 

tn 

o,v_-ic!  a 

a      a  "C? 

(N 

i>-lS^ 

p.    0~_- 

-   bD«   ..IT 

T3I-H 

llet 

ndin 

VII 

erve 

trac 

3 

JB  s,5  a 

Ph  0 

3 

3 

-^■^  a  3 
,2  "^  o  fl 

"^    ID'S 


s  s'""'' 


ac 


o£-« 


eg 

3^ 


ca.2  I 
a  60^ 
i2  c! 


03 


IP 


C?  fi 


'a^ 


^a 


^:g 

1' 

m   0 

ereb 
spin 
des( 
mbi 

U 

a  n, 
3J3 


--^G-SB-lZ' 


X 


a)  > 
3  a 


5;  6 


^  03 


O  ® 

X!  a 


Hi    H. 


«.2 


3  55 

a 


|«co 


to  ii  o 

==  S  ft 


.SPS^ 


!5 

a'3  3 

(^  q  0) 

CO    3     -     . 


S-S  3 
3   rt   S   hn 


bO 


PONTINE  SYNDROMES 

a' 


395 


Fig.  200. — Posterior  (caudal)  pontine  lesion.  Contralateral  anesthesia  with  alternate 
paralysis  of  the  VI,  VII,  VIII  pair  and  of  the  spinal  vagus  (Xa)  from  a  tubercle  in  the 
inferior  (caudal)  portion  of  the  left  pontine  tegmentum.  The  tubercle  has  pressed  upon 
or  destroyed  the  secondary  crossed  paths  of  the  tegmentum  (SR) ,  the  median  lemniscus 
(Rm) ,  the  posterior  longitudinal  fasciculus  (Flp) ,  the  nuclei  and  root  fibers  of  the  external 
rectus,  of  the  facial,  the  vestibular,  and  the  cochlear  branches  of  the  eighth. 

On  the  right  there  is  hemianesthesia  of  the  body  and  of  the  face  for  all  modes  of  deep 
and  superficial  sensibility.  Cerebellar  hemiataxia  and  lateropulsion  by  lesion  of  the 
cerebellar  pathways. 

On  the  left  there  is  atrophic  facial  paralysis,  (a)  superior  facial,  inferior  of  the 
platysma,lagophthalmia,  drooping  mouth  angle,  cochlear  deafness,  vestibular  nystagmus, 
convergent  strabismus,  paralysis  of  the  lateral  associated  movements  of  the  eyes  toward 
the  left  by  lesion  of  the  internu clear  and  vestibular  left  oculorotary  fibers.  By  reason 
of  the  predominance  of  the  antagonists  the  patient  looks  to  the  right.  There  is  a  velo- 
palatine  paralysis  and  a  paralysis  of  the  vocal  cords  (nuclei  and  root  fibers  of  the  spinal 
vagus) .  Hemianesthesia  of  the  face  by  lesion  of  the  descending  root  of  the  trigeminus 
(a')-    (Dejerine.)     (See  Chart,  No.  14,  p.  392.) 


396     DISEASES  OF  THE  PONS,  BRAIN  STEM,  AND  MIDBRAIN 


S  s 

3  o 


5°   ^i   ^ 


Ft) 


_a>-c 


J.  i.i,  g  b 

a  ^  is  "  t.  o 
"„  rt  "3  =1  c 

Q^  C3    »>1   ^  -4^  -tJ 


moo.    -.2-2 
'S  "'3.2  M  o 

.r?--5  s  £  fe  >> 

p,  >       a;  § 


t;'     _  c  c  a  d 


a?'2       is"  N       is  S       «8  i       is" 
§:JS     'Si)  S,     "SI  S,     'mi  n.    'm 


'm^3 

0)  cS  o 

■^  h  o 


S  <o  a     ■-  >, 


'  o  o. 
>>'m;=J 

■a  >  -- 


—  j3  a) 


>-  0)^  S—  a+^^  S— I  a-S  ?,^ 

^b[iS_>Ht.ftS       t^  u.  a  ^  p. 


—,  o     — I  o 


g  cS  S  ^  I 


«  a.i 


5  OJ  d  -^^ 
to  01^   o 


*-JJ 

CO 

(h 

t4 

,_^ 

^ 

>.T! 

^ 

cS 

CI 

K! 

01 

0) 

rt 

c3 

i 

f^ 

o 

03 

o3 

03 

^'f. 

_g 

4-> 

a 

fi 

a 

>?fi 

Pi 

> 

c1  O 

U 

U 

U 

J!h  S'S  S.S  "■^■p 

Bo30t,^g^B 
+jt,c3cS-SKffl 


?^rl 


5S 


■S^ 


ti 

XI 

O  -IJ 

« 

> 

^_, 

o 

;5   S" 


«1 


S  feS  all 

^-5.2 

ucl 
I  n 

(2) 
liar 
orm 

!^  ^"-S  ^ 

m -a  t. 

«illl 

«  °*  g  g"-^ 

03  i:-T3 

^•^a^'fc  "^ 

^^  a  ■* 

^-^ 

SSfe 


>>«    -       ^ 


.« 


T3  o 
5  o 


bj)^ 

co^ 

o  .- 

^1 

-f  {3 

S^J 

ft3  « 

«  ag 

03  S 

ro'3 

:wj5> 

^go 

>>T>2 

>>"E  a 

fii^^ 

---.Sw 

«      H 


03  _  — , 

'm  oj  03 

^  m  PQ 


cq"n 


-a         -;3  -73 


PONTINE  SYNDROMES 


397 


O.e 


Fig.  201. — Anterior  pontine  syndrome.  Inferior  alternate  paralysis — Millard- 
Gubler  type.  Contralateral  hemiplegia  of  the  trunk  and  of  the  extremities  of  the  ponto- 
meduUary  type  (without  inferior  facial  palsy)  without  hemianesthesia,  and  without 
the  syndromes  of  Foville,  with  alternate  paralysis  may  be  of  the  sixth  nerve  alone  (a) 
or  of  the  sixth  and  seventh  (a  and  b) .  At  this  level  the  cortico-nuoleo-f acial  fibers  have 
left  the  pyramidal  tract  with  the  meduUopontine  aberrant  fibers  (see  Plate  II)  and 
are  in  the  tegmentum,  hence  the  absence  of  the  inferior  contralateral  facial  palsy. 

At  right  there  is  hemiplegia  of  the  extremities  without  paralysis  of  the  inferior  facial. 

To  the  left  there  is  convergent  interna)  strabismus  by  paralysis  of  the  external  rectus ; 
the  predominance  of  the  antagonists  carries  the  eye  to  the  left  and  up.  There  is  no 
forced  position  of  the  head,  the  associated  lateral  movements  of  the  eyes  being  possible, 
only  the  left  eye  is  involved. 

In  (b)  there  is  added  to  the  preceding  an  atrophic  facial  paralysis  with  RD  (VII). 
The  facial  folds  are  gone,  there  is  lagophthalmus,  drooping  of  the  angle  of  the  mouth  and 
paralysis  of  the  elevators.     (Dejerine.)     (See  Chart,  No.  15,  p.  392.) 


398     DISEASES  OF   THE  PONS,  BRAIN  STEM,   AND  MIDBRAIN 
a  b 


Fig.  202. — Pontine  syndrome.  Millard-Grubler-Foville  syndrome.  Hemiplegia  of 
cerebral  type  with  hemianesthesia  of  the  extremities  and  of  the  head  of  the  side  opposite 
and  alternate  paralysis  may  be  of  the  (VI)  only  (a)  or  of  the  VI  and  VII,  (b)  by  throm- 
bosis of  the  inferior  portion  of  the  basilar  artery,  in  particular  of  the  median  pontine 
arteries  of  the  left  side.  The  lesion  occupies  the  anterior  part  of  the  pons,  destroys 
the  pyramidal  tract  (Py)  extends  to  the  tegmentum,  sections  the  median  lemniscus 
fibers  (Rm),  the  fibers  of  the  reticular  formation,  the  posterior  longitudinal  fasciculus 
(Flp),  the  root  fibers  of  the  VI  pair.  In  (6)  the  more  extensive  lesion  destroys  in  addi- 
tion the  root  fibers  of  the  facial  and  the  nuclei  of  the  VI  and  VII  and  the  fibers  of  the 
lateral  portion  of  the  reticular  formation. 

On  the  right  there  is  contralateral  hemiplegia  of  the  cerebral  type — ^extremities  and 
inferior  facial,  by  lesion  of  the  corticospinal  pyramidal  fibers  and  of  the  cortico-nucleo- 
f acial  (aberrant-medullo-pontine  fibers) .  Hemianesthesia  of  the  extremities  and  of  the 
face  in  (a)  involving  particularly  the  tactile  sensibility  and  postural  sense,  and  in  (b) 
all  modes  of  sensibility,  these  being  more  marked  in  the  head  than  in  the  extremities. 

On  the  left  side  there  is  paralysis  of  the  external  rectus  (VI)  internal  strabismus,  to 
which  there  is  added  in  (b)  a  paralysis  of  the  facial  (VII)  inferior,  superior,  and  of  the 
platysma  myoides,  lagophthalmia,  drooping  of  the  angle  of  the  mouth,  flattening  of 
the  facial  lines. 

Furthermore,  there  is  paralysis  of  lateral  movements  of  the  eyes  toward  the  left  by 
lesion  of  the  left  posterior  longitudinal  fasciculus  (Flp) .  By  reason  of  the  predominance 
of  the  antagonists  the  patient  looks  toward  the  right.  (Dejerine.)  (See  Chart,  No.  16,  p, 
392.) 


CHAPTER  IX. 
CEREBELLAR  SYNDROMES. 

Defect  or  disorder  of  the  cerebellum  itself,  or  of  its  chief  afferent 
and  efferent  paths  gives  rise  to  a  number  of  fairly  definite  syndromes. 
These  may  be  referred,  with  a  certain  degree  of  accuracy,  to  the 
structures  involved.  There  are,  furthermore,  other  disturbances,  the 
precise  nature  of  whose  mechanisms  is  still  unsatisfactorily  analyzed, 
although  it  is  recognized  that  cerebellar  mechanisms  are  involved. 

These  syndromes  may  be  the  result  of  defect  or  disorder  of  the 
organ  itself,  or  of  its  connections,  or  they  may  be  due  to  or  compli- 
cated by  the  position  that  the  cerebellum  itself  occupies  with  refer- 
ence to  contiguous  structures  in  the  posterior  cerebral  fossa. 

The  cerebellum  occupies  the  posterior  cerebral  fossa,  is  separated 
from  the  occipital  lobes  of  the  cerebrum  by  the  tentorium,  and  rests 
upon  the  pons  and  medulla,  forming  part  of  the  upper  boundary  of 
the  fourth  ventricle.  It  is  connected  with  the  rest  of  the  nervous 
system  by  the  anterior  medullary  velum,  the  superior,  middle,  and 
inferior  cerebellar  peduncles  and  posteriorly  by  the  posterior  medullary 
velum.  The  tela  choroidea  forms  the  posterior  continuation  of  this 
latter  structure,  and  serves  as  a  roof  to  the  posterior  part  of  the  fourth 
ventricle. 

Being  so  intimately  connected  with  structures  in  the  midbrain, 
the  red  nucleus  and  the  optic  thalamus,  with  bulbar  and  pontine 
centers  and  with  the  cord ;  lying  above  important  structures,  and  con- 
taining important  nuclei,  the  dentate  nucleus,  Deiters'  nucleus,  nucleus 
globosus,  nucleus  emboliformis,  tectal  nuclei,  etc.;  with  a  multiplic- 
ity of  afferent  and  efferent  tracts,  the  possibilities  of  symptomatology 
are  very  numerous. 

The  cerebellum  is  the  central  organ,  composed  of  groups  of  centers, 
for  the  coordination  of  the  reflex  system  of  the  proprioceptors;  that 
is,  those  sensory  impressions  coming  from  receptors  throughout  the 
entire  body.  It  thus  represents  the  entire  body.  These  receptors 
receive  impressions  of  thermal,  tactile,  gravity,  weight,  pain,  sound, 
light,  chemical  and  other  stimuli,  and  by  means  of  afferent  paths, 
transmit  them  chiefly  to  the  cortex  of  the  cerebellum.  Many  of  these 
paths  are  definitely  known;  others,  particularly  those  connected  with 
the  viscera,  are  still  under  investigation.^ 

From  the  cerebellar  cortex,  which  may  thus  be  looked  upon  as 
chiefly,  if  not  exclusively,  sensory,  these  impulses  pass  to  the  various 
nuclei  of  the  cerebellum,  and  are  there  redistributed.    It  would  appear 

1  Bechterew,  Die  Funktiouen  der  Nerveucentra,  1909,  ii. 


400 


CEREBELLAR  SYNDROMES 


that  these  intrinsic  cerebellar  nuclei  are  mainly  motor.^  The  move- 
ments of  the  head  being  chiefly  referable  to  the  intrinsic  nuclei,  those 
of  the  trunk  and  limbs  to  the  paracerebellar  nuclei. 

The  chief  afferent  or  receptor  paths  arrive  by  way  of  the  three 
cerebellar  peduncles. 

Bechterew  (loc.  cit.)  describes  seven  paths  as  passing  through  the 
inferior  cerebellar  peduncle,  or  restif orm  body.    These  are  in  part : 

1.  The  dorsospinocerebellar  tract  of  Flechsig,  which  passes  up  the 
lateral  ventral  side  of  the  lateral  column,  originating  from  cells  in 
Clark's  column,  from  the  upper  lumbar  to  the  upper  dorsal  segments. 
This  tract  passes  up  through  the  inferior  cerebellar  peduncle  (corpus 


Fig.  203. — Hemorrhage  of  pons. 


restif orme),  and  is  thought  to  be  distributed  to  the  middle  lobe  of 
the  vermis  and  the  ventrolateral  lobe  of  the  lobus  centralis^  (Mott). 

2.  The  posterior  columns  of  Goll  and  Burdach  send  fibers  from 
their  nuclei  in  the  medulla  by  way  of  the  restiform  body,  dorsally 
and  uncrossed  to  the  inferior  vermis,  ventrally  and  crossed  to  the 
superior  vermis.  (Many  authors  claim  that  these  bundles  have  no 
connection  with  the  cerebellum.) 

3.  The  olivocerebellar  tract,  which  originates  in  the  cord,  ends  in 
the  inferior  olive,  from  which  it  passes  direct  (?)  to  Deiters'  nucleus, 
and  crosses  to  the  superior  vermis. 

1  Horsley,  Functions  of  the  Cerebellum,  Brain,  1906,  xxix,  446. 

2  Monatsschrift,  1891,  i,  104. 


CEREBELLAR  SYNDROMES  401 

4.  Vestibulocerebellar  path  from  the  vestibular  ganglion,  which 
sends  its  central  fibers  to  the  nucleus  vestibularis,  and  to  Deiters' 
nucleus  in  the  tegmentum,  and  from  thence  to  the  inferior  vermis. 
(The  details  of  these  pathways  are  given  in  the  chapter  on  the 
vestibular  functions,  also  in  Plates  X  and  XI.) 

These  pass  up  through  the  lateral  part  of  the  restiform  body.  In 
the  median  portion  there  are  two  groups  of  fibers:  One  contains 
sensory  fibers  from  the  cranial  nerves,  the  trigeminus  and  vestibular. 
They  form  the  direct  cerebellar  sensory  path  of  Edinger.  Other  fibers 
connect  the  nuclei  of  the  cranial  sensory  nerves  to  the  cerebellum. 
Both  of  these  bundles  end  for  the  most  part  in  the  tegmental  nuclei 
This  tractus  nucleocerebellaris  is  an  indirect  path.^ 


Fig.  204. — Hemorrhage  of  pons. 

In  the  middle  cerebellar  peduncle  incoming  fibers  come  from  the 
nuclei  of  the  pontine  reticular  formation  and  the  ventrolateral  pontine 
nuclei.  Certain  of  these  fibers  are  in  relation  to  fibers  coming  from 
the  frontal  area  to  the  pontine  nuclei  and  form  part  of  a  frontocerebellar 
reflex  path.    (See  Fig.  205.) 

The  fibers  passing  to  the  cerebellum  by  means  of  the  superior 
cerebellar  peduncle  are  four  in  number,  according  to  Bechterew.  The 
best  known  of  these  is  Gowers'  tract,  which  passes  into  the  cerebellum 
by  means  of  the  superior  cerebellar  peduncle.  According  to  Edinger 
and  Horsley  this  tract  is  distributed  to  the  entire  cerebellar  cortex. 
Fibers  from  the  thalamus,  from  the  red  nucleus,  and  collaterals  from 
the  nuclei  of  the  eye  muscles  have  also  been  traced  through  the 
superior  cerebellar  peduncles. 

1  Bechterew,  ii,  961. 
26 


402 


CEREBELLAR  SYNDROMES 


Afferent  Tracts. — It  is  also  through  the  superior  cerebellar  peduncles 
that  the  chief  connections  between  the  cerebellum  and  the  sensori- 
motor areas  of  the  cerebrum  are  carried.  These  cerebellorubral  and 
cerebellothalamic  fibers  originate  in  the  dentate  and  other  cerebellar 


Fig.  205. — The  fiber  tracts  of  the  posterior  cerebellar  peduncle,  ra,  anterior  roots; 
rp,  posterior  roots;  /c,  posterior;  fc',  ventral  cerebellar  tracts;  fi,  aberrant  pyramidal 
fibers;  fG,  column  of  Goll;  fB,  column  of  Burdach;  fd,  descending  tract  from  Deiters' 
nucleus;  V/J/?;,  vestibularis;  ^,  fibers  from  lateral  nuclei  of  medulla;  /grc, /6c,  cerebellar 
fibers  from  posterior  column  nuclei;  pv,  vpi,  latero ventral  and  dorsomedian  pontine 
nuclei;  nD,  Deiters'  nucleus;  nv,  vestibularis  nucleus;  d,  descending  bundle  of  median 
portion  of  posterior  cerebellar  peduncle;  a,  ascending  fibers  of  vestibularis;  nd,  tectal 
nuclei;  ce,  descending  path  from  tectal  nuclei  to  superior  olive;  cs,  superior  cerebellar 
peduncle;    rn,  nucleus  retrolenticularis;    fl,  fibers  from  Deiters'   nucleus  to  posterior 


longitudinal  bundle 
cerebellar  peduncle 


OS,  superior  olive;   fa,  fibers  from  flocculus  to  vermis;    cm,  median 
fv,  fasciculus  verticalis  pontis;    fct,  central  tegmental  tract;    oi. 


inferior  olive;  fo,  olivary  fasciculus.     (Bechterew.) 


nuclei,  and  passing  contralaterally  to  the  red  nucleus  and  thalamus, 
end  there  to  be  continued  further  to  the  cerebrum. 

The  cerebellum  therefore  forms  an  important  sensorimotor  station 
in  a  complicated  series  of  reflexes  which  work  for  the  most  part  auto- 
matically.   The  cerebellar  cortex  acts  as  the  primary  reception  center, 


FiQ  206  —The  ascending  cerebellar  tracts  of  the  spinal  cord  and  their  central  path- 
ways" B,  Burdach's  column;  ca,  anterior  cerebellar  fasciculus  to  the  vermis  through  the 
anterior  medullary  velum  co,  cerebello-olivary  fibers;  cp,  common  path  from  posterior 
column  nuclei  to  vermis;  d,  dentate  nucleus;  fi,  internal  arcuate  fibers;  /p,  posterior 
arcuate  fibers;  ft,  fibers  from  vermis  cortex  to  tegmental  nuclei;  G,  column  of  Goll; 
a  corticovermis  association  fibers;  gl,  nucleus  globosus;  p,  nucleus  emboliformis; 
rv,  posterior  sensory  root  fibers;  sc,  subcortical  paths  of  the  brachiumconjunctivum; 
so,  olivospinal  tract;  t,  tegmental  nuclei;  tr,  fasciculus  spinocerebellaris ;  Vi,  inferior 
vermis;  Vs,  superior  vermis.     (Bechterew.) 


404 


CEREBELLAR  SYNDROMES 


the  impulses  coming  from  the  cord  and  brain  stem  traversing  the 
paths  just  enumerated,  giving  information  chiefly  concerning  the 
states  of  tension  of  the  muscular  apparatus  of  the  trunk,  the  extremi- 
ties, and  the  head,  and  the  states  of  pressure  in  the  joints  and  tendons. 
The  reflexes  combine  to  regulate  the  constantly  altering  positions  of 


Fig.  207. — Descending  pontine  and  cerebellar  tracts:  VI,  root  fibers  abducens;  cp, 
corticopontine  fibers;  crp,  fibers  from  pons  to  cerebellum;  d,  dentate  nucleus;  fed, 
cerebello-Deiters'  fibers;  fo,  tegmento-olivary  fibers;  ft,  fibers  from  vermis  to  tegmental 
nuclei;  g,  cortical  association  fibers;  NVI,  abducens  nucleus;  nd,  Dieters'  nucleus; 
Nol,  inferior  olive;  nt,  tegmental  nuclei;  os,  superior  olive;  ra,  anterior  root  fibers. 
(Bechterew.) 

the  entire  body  in  space,  and  possibly  some  of  the  viscera.  The 
cerebellum  therefore,  in  this  sense,  acts  as  a  regulating,  coordinating 
organ  for  the  estimation  of  the  body  in  space.  To  the  spinal,  midbrain, 
cerebral  reflex  arcs  there  are  also  added  fronto-pyramido-ponto-bulbo- 
cerebellar  arcs  which  contain  involuntary  as  well  as  voluntary  regula- 


SYMPTOMS 


405 


tory  coordinating  impulses,  acting  to  orient  the  body  in  practically 
all  of  its  spatial  relations.  Cyon  adds  that  therefore  the  cerebellum 
is  intimately  concerned  with  the  mathematical  sense  in  its  objective 
space  relations.^ 

Symptoms. — The  general  symptoms  of  cerebellar  disease  therefore 
offer  a  multitudinous  complex,  the  details  of  which,  and  their  anatomo- 
pathological  correlations,  are  still  being  rapidly  added  to.  Among 
these,  however,  there  are  certain  cardinal  types  which  are  capable 
of  partial  analysis  at  the  present  time,  and  to  which  attention  will 
be  directed. 


Figs.  208  and  209. — -The  peculiar  stand  and  gait  of  cerebellar  ataxia.     (Thomas.) 


Cerebellar  Ataxia. — This  complex  of  disturbances  is  one  of  the 
classical  signs  of  cerebellar  disorder.  It  consists  in  a  disturbance  of 
the  coordination  of  the  larger  muscular  group  actions,  principally^  of 
the  trunk  and  lower  extremities,  although  the  head,  eyes,  and  upper 
extremities  are  not  uninvolved.  Thus,  standing,  walking,  and  the 
making  of  fine  coordinated  movements  are  interfered  with  in  the 
absence  of  signs  of  distinct  paralysis. 

In  walking,  the  wobbling,  side-stepping  gait,  so  well  described  by 
early  French  authors  (Duchenne,  of  Boulogne)  as  the  drunken  gait, 
is  characteristic.     Incidentally  it  may  be  added  they  are  identical. 


'  Cyon,  Das  Ohrlabyrinth,  1908. 


406 


CEREBELLAR  SYNDROMES 


since  alcohol  dissociates  the  sensory  neurons  by  raising  the  synaptic 
junction  threshold.  In  severe  grades  of  cerebellar  ataxia  standing, 
and  even  sitting,  becomes  impossible.  In  milder  grades  one  sees  the 
same  type  of  disturbance  in  many  choreas,  in  paralysis  agitans,  in 
general  paresis,  multiple  sclerosis,  etc. 

A  partial  study  of  the  cerebellar  gait  has  shown  two  characteristic 
trends  of  disturbance:^  first,  staggering  (lateropulsions)  toward  the 
aiTected  side,  at  times  forward  or  backward,  according  to  location  of 
lesion  in  vermis  (or  dentate  nucleus).  The  patient  feels  as  though 
shoved  to  one  side  and  in  the  attempt  at   restitution   overcorrects 

(asynergia  of  Babinski)  and  thus  sways; 
second,  the  entire  orientation  in  space 
is  influenced  and  the  patient's  move- 
ments, as  a  whole,  swerve  in  the  direction 
of  the  affected  side.  (Menagery  move- 
ments, as  seen  in  whirling  white  mice; 
tumbling  movements,  as  seen  in  the 
tumbler  pigeon.) 

Conscious  attempts  at  correction 
(frontocerebellar  paths)  produce  the 
larger  zig-zags  in  the  general  course  of 
the  progression.  Forward  and  back- 
ward movements  have  their  special 
localizing  signs  to  be  spoken  of.  From 
the  anatomical  considerations  these 
ataxias  may  result  from  involvement 
of  the  spinocerebellar  paths  (Flechsig, 
Gowers — vestibular  systems),  as  in  the 
Friedreich  and  Marie  ataxia  group; 
from  involvements  in  the  cerebellum 
itself  (tumors,  cysts,  ageneses,  scleroses) ; 
in  affections  involving  the  superior  cere- 
bellar peduncles — from  bulbar  and  pon- 
tine involvements  of  these  paths,  and 
also  from  implication  of  the  cerebello- 
rubral, cerebellothalamic,and  frontocere- 
bellar paths.  Typical  cerebellar  ataxias  are  thus  seen  in  some  frontal 
tumors,  with  classical  intoxication  gait. 

Chiefly  associated  with  ataxias  of  the  trunk  and  the  lower  extremities 
in  standing,  walking,  etc.,  are  the  bilateral  or  unilateral  (usually  homo- 
lateral) ataxias  (dysmetrias)  of  the  upper  extremities.  The  individual 
muscular  activities  are  illy  coordinated,  botb  as  to  space  and  time, 
the  desired  act  only  being  arrived  at  after  several  trials  and  errors, 
as  in  walking.  Visual  aid,  or  its  lack,  has  little  or  no  influence  on  the 
ataxia,  as  tested  by  the  finger-nose  test  and  finger-finger  test,  and  the 


Fig.  210. — ^Cerebellar  gait  attitude. 
(Thomas.) 


1  Stewart  and  Holmes,  Brain,  1904. 


SYMPTOMS 


407 


ataxia  is  a  constant  one,  non-increasing,  as  in  an  intention  tremor 
(with  varying  graduations,  for  certainly  the  intention  tremors  of  mul- 
tiple sclerosis  are  often  due  to  interference  with  cerebellar  mechanisms). 


J 

Fig.  211. — Test  for  dysmetria  in  cerebellar  disturbances. 


Fig.  212. — Showing  cerebellar  dysmetria. 


Adiadokolxinesis. — Originally  described  by  Babinski  as  character- 
istic of  cerebellar  disease,  this  symptom,  consisting  of  a  disability  in 


408  CEREBELLAR  SYNDROMES 

the  performance  of  rapid  movements  involving  the  alternating  actions 
of  agonist  and  antagonist  muscles  (see  Examination),  is  not  invariably 
found  nor  always  clearly  indicative  of  cerebellar  disorder;  yet  it  is 
so  frequently  found  as  to  merit  special  attention.  It  is  in  part  a  variant 
of  ataxia  brought  out  by  a  special  test.  Here  the  timing  sense  that 
is  regulated  by  the  cerebellum  is  at  fault.  In  the  absence  of  paralysis 
it  usually  is  indicative  of  cerebellar  path  involvement.  It  is  frequently 
absent  in  extracerebellar  tumors  in  which  there  are  other  well-marked 
cerebellar  signs,  as  in  frontal  tumors. 

Vertigo. — ^As  the  chief  organ  of  orientation  in  space,  severe  disturb- 
ance of  certain  of  the  cerebellar  reflex  paths  causes  vertigo,  which 
is  apt  to  be  a  prominent  and  a  fairly  constant  sign.  The  vertigo  is 
of  a  rotatory  character.  The  patient  may  not  only  feel  himself  revolv- 
ing in  space,  but  objects  may  go  around  from  right  to  left  or  from  left 
to  right;  more  rarely  the  vertigo  has  an  up  or  down  character.  Each 
of  these  two  characters  is  to  be  closely  inquired  into.  Here  the 
chief  lesions  are  connected  with  the  vestibular  paths,  as  the  labyrinth 
is  the  chief  cephalic  ganglion  in  the  whole  proprioceptive  system,  of 
which  the  cerebellum  constitutes  the  coordinating  center.  Thus 
labyrinthine  disease  itself,  as  well  as  disease  of  its  extracerebellar  or 
intracerebellar  paths  may  give  rise  to  the  symptom.  By  means  of 
the  specific  tests  devised  by  Barany  (see  chapter  on  Examination)  a 
separation  of  labyrinthine  diseases  of  extracerebellar  origin  is  usually 
possible. 

Further,  enough  experience  has  accumulated  (Stewart  and  Holmes, 
loc.  cit.)  to  show  that,  in  general,  objects  rotate  from  the  diseased  to 
the  well  side  for  intracerebellar  as  well  as  extracerebellar  affections, 
whereas  the  subjective  sense  of  rotation  is  usually  from  the  diseased 
to  the  well  side  in  intracerebellar  involvement,  and  the  reverse  in 
extracerebellar  involvement  of  the  paths.  This  generalization,  a 
little  too  broad,  is  in  need  of  further  study,  and  of  more  accurate 
localization. 

Nystagmus. — ^A  fourth  sign,  rarely  absent  in  cerebellar  path  dis- 
turbance, is  nystagmus.  It  is  also  closely  related  to  the  vestibular 
reflex  system,  and  may  result  from  extracerebellar  involvement  as 
well  as  intracerebellar  implication  of  the  paths. 

True  vestibular  nystagmus  is  almost  invariably  accompanied  by 
vestibular  vertigo  and  ataxia.  Vestibular  nystagmus  itself  is  usually 
modified  by  the  position  of  the  head,  hence  every  person  with  nys- 
tagmus must  be  examined  with  the  head  in  the  three  planes;  a  patient 
with  vestibular  nystagmus  tends  to  rotate  within  the  plane  of  the 
nystagmus,  and  in  the  direction  opposite  to  that  of  the  quick  nystagmus 
movement. 

A  patient  with  vestibular  nystagmus  then,  who  bends  his  head  for- 
ward at  90  degrees  will  rotate  in  a  direction  directly  opposite  if  he  bend 
his  head  backward  90  degrees.  The  laws  of  intracerebellar  nystagmus, 
apart  from  actual  vestibular  disease,  remains  to  be  investigated  (con- 


SYMPTOMS 


409 


jugate  deviations,  skew  deviations,  and  other  eye  displacements  are 
to  be  interpreted  in  the  light  of  forced  movements  having  their 
analogies  to  nystagmus,  and  are  considered  in  the  chapter  on 
Midbrain  'Disease.  See  illustrations  of  conjugate  palsies;  also  in 
chapter  on  the  Eye  Paths.  See  plate  of  oculorotary  and  cephalorotary 
mechanisms  (Plate  VIII). 

Closely  associated  with  disorder  of  the  vestibular  system  are  pain 
in  the  muscles  of  the  neck,  nausea,  vomiting,  amblyopias,  and  loss  of 
consciousness. 


Fig.  213. — Asynergia  of  Babinski  devel- 
oped on  attempting  to  take  hold  of  a  glass. 
The  fingers  are  held  very  far  open. 
(Thomas.) 


Fig.  214. 


-Asynergia  of  Babinski. 
(Schaller.) 


Cerebellar  Hypotonus. — Palpation  of  the  muscles,  testing  of  resis- 
tance movements,  and  looseness  on  the  performance  of  passive  move- 
ments, reveal  a  type  of  muscular  hypotonus  in  cerebellar  affections 
which  is  usually  homolateral.  This  hypotonia,  or  atonia,  is  usually 
accompanied  by  normal  or  even  exaggerated  tendon  reflexes  in  con- 
trast to  that  of  peripheral  neuritis  or  tabes.  One  feature  of  this 
hypotonus  noted  by  Stewart  and  Holmes  is  striking.  If  a  resistance  to 
a  definite  movement  be  suddenly  relaxed,  in  the  normal  flexing  of  the 
arm,  for  instance,  there  is  a  sudden  flexor-jerk,  followed  by  an  extensor 


410  CEREBELLAR  SYNDROMES 

recoil.  In  a  cerebellar  hypotonic  reaction  the  flexor-jerk  is  excessive, 
and  is  rarely  followed  by  a  recoil. 

Asthenia. — A  paresis  or  asthenia,  usually  homolateral,  is  closely 
related  to  cerebellar  hypotonus.  Its  presence  in  cerebellar  disorder 
has  usually  been  interpreted  as  due  to  a  lesion  of  the  pyramidal  tracts 
by  contiguity;  unquestionably,  however,  it  is  a  true  cerebellar 
symptom.  It  is  chiefly  present  when  the  cerebello-vestibulo-spinal 
and  rubrospinal  tracts  are  affected.  Since  the  rubrospinal  tract  is  to  be 
interpreted  as  an  auxiliary  to  the  pyramidal  tract,  the  different  opinions 
of  various  authors  may  find  a  common  adjustment. 

Cerebellar  Asynergia. — Described  by  Babinski^  as  a  special  symptom 
of  cerebellar  disease,  this  symptom  is  in  reality  one  of  the  components 
of  cerebellar  ataxia,  but  in  the  anteroposterior  plane,  rather  than  in 
in  the  lateral  planes.  It  consists  in  the  patient's  inability  to  balance 
himself,  whereby  his  legs  either  walk  away  from  under  him,  or  he 
pitches  forward  without  their  following.  It  is  a  severe  grade  of  retro- 
pulsion  and  propulsion,  as  seen  in  paralysis  agitans,  and  due  in  both 
instances  to  similar  pathological  foundations. 

Cerebellar  Fits. — Originally  described  by  Jackson^  as  tetanus-like 
convulsive  seizures,  with  characteristic  holding  of  the  body  in  extended 
rigid  position. 

Forced  Movements. — These  are  present  in  the  neck  muscles,  muscles 
of  the  eyes,  and  appear  as  irritative  or  as  defect  symptoms,  due  to 
disease  of  the  hemispheres  or  of  the  middle  cerebellar  peduncle.  (See 
Diseases  of  Midbrain.) 

Speech  Disturbances. — Dysarthrias  usually  indicate  the  same  type  of 
ataxia  as  found  in  other  muscles  of  the  body,  adiadokokinesis.  They 
are  usually  present  with  defects  of  the  cerebellum,  and  may  indicate 
general  defect  of  the  entire  apparatus,  disease  of  the  bulbocerebellar 
tracts  or  pressure  upon  the  bulbar  nuclei  from  contiguous  new  growths. 

Chief  Syndromes. — In  discussing  disorders  of  the  cerebellum  it  is 
convenient  to  take  up  first  affections  of  the  peduncles,  although  very 
rare,  then  of  the  cerebellum  itself,  and  finally  diseases  of  intracere- 
bellar  location,  which  latter  occupying  the  posterior  fossa  implicate  the 
cerebellar  mechanisms,  and  those  of  its  contiguous  structures,  the  pons, 
medulla,  and  fourth  ventricle. 

Inferior  Cerebellar  Peduncle — Corpus  Restiforme. — Isolated  disease  of 
this  structure  is  rare.  Pressure  due  to  bulbar  and  pontine  disease  is 
not  uncommon.  The  most  characteristic  symptoms  are  modifications 
of  the  eye  movements,  convergent  and  divergent  strabismus,  even 
skew  deviation,  forced  positions,  turning  of  body  toward  the  site  of 
lesion,  and  vertigo,  with  tendency  to  fall  in  the  direction  of  the  side 
of  the  lesion.  The  chief  mechanism  involved  is  the  cerebello-vestibulo- 
spinal  tract. ^     (See  Midbrain  Lesions,  14,  19,  21,  26,  etc.) 

1  Rev.  Mens.  Int.,  May,  1909. 

2  British  Med.  Jour.,  November  4,  1871.     Reprint,  Brain,  1906,  p.  425. 

^  Adler,  Die  Symptomatologie  der  Kleinhirnerkrankungen,  Wiesbaden,  1899,  has 
collected  the  cases. 


APLASIAS  OF   CEREBELLUM  411 

Lesions  of  the  Middle  Cerebellar  Peduncle. — These  cause  rolling  move- 
ments of  the  body  on  its  vertical  axis,  skew  deviation  of  the  eyes, 
Magendie-Hertwig  syndrome,  consisting  in  one  eye  being  higher  than 
its  mate.  The  patients  behave  as  though  they  had  bilateral  vestibular 
disease,  causing  the  rolling  motions;  the  eye  symptoms  depend  on 
lesions  of  the  fasciculus  anteromarginalis,  fibers  to  the  posterior  longi- 
tudinal bundle,  and  interference  with  the  fibers  to  the  abducens 
nucleus. 

There  are  few  uncomplicated  cases  on  record.  Pontine  lesions 
often  give  rise  to  symptoms  from  implication  of  the  middle  peduncle. 
(See  Midbrain  Syndromes.) 

Lesions  of  the  Superior  Cerebellar  Peduncles. — Isolated  lesions  of  these 
peduncles  are  rare.  The  symptoms  are  usually  choreic,  or  paralysis 
agitans-like  tremors  on  the  same  side  of  the  lesion — possibly  due  to 
implication  of  the  cerebello-rubro-spinal  bundle  in  the  tractus  cerebello- 
tegmenti.  Forced  positions  of  the  head  to  the  side  of  the  lesion  have 
been  described.  Ocular  implications  rarely  occur,  although  nystagmus 
has  been  observed.     (See  Midbrain  Lesions.) 

Lesions  of  Cerebellum  Itself. — The  most  important  of  these  are  age- 
neses  or  aplasias,  scleroses  or  atrophies,  hemorrhage,  softening,  inflam- 
mation, abscesses,  and  tumors. 

Aijlasias  of  Cerebelluin. — These  are  congenital,  and  represent  a 
vast  array  of  different  conditions;  total  lack  of  cerebellum,  absence 
of  the  lateral  (in  old  sense)  lobes,  absence  of  vermis,  unilateral  loss, 
irregular  defects,  and  general  congenital  smallness  of  the  cerebellum 
and  cerebrum.  A  consistent  symptomatological  grouping  is  not  yet 
possible.  Mingazzini^  has  attempted  it.  With  the  newer  studies  in 
localization  by  Horsley,  Bolk,  and  others  the  entire  study  of  cerebellar 
representation  will  see  marked  advance  in  the  near  future. 

Mingazzini's  grouping  of  the  conditions  is  as  follows: 

1.  Pure  unilateral  ageneses  and  atrophies. 

2.  Pure  bilateral  ageneses  and  atrophies. 

3.  Cerebellar  atrophies  associated  with 

(a)  Disease  of  the  cerebrum. 
(6)  Disease  of  the  spinal  cord. 

1.  Unilateral  loss  of  a  lateral  (in  old  sense)  lobe  may  be  present 
without  any  symptoms  according  to  present  developed  modes  of 
testing.  Few  of  these  cases  have  been  tested  by  more  recent  methods. 
In  certain  instances  unilateral  atrophy  has  been  associated  with 
epileptiform  convulsions,  or  retropulsion,  when  the  superficies  of  the 
affected  hemisphere  is  atrophic;  if  the  atrophy  involves  a  part  of  the 
vermis,  slight  motor  signs,  such  as  slowing  of  the  gait,  have  been 
observed. 

2.  Pure  Bilateral  Agenesis. — In  some  of  the  cases  reported  no  symp- 
toms have  been  observed  (old  cases).    The  commonest  symptoms  are 

1  Monatsch.  f.  Neur.  u.  Psych.,  1906,  xviii,  76. 


412 


CEREBELLAR  SYNDROMES 


Fig.  215. — Pontine  middle  syndrome.  Lesion  of  the  lateral  part  of  the  left  pontine 
tegmentum,  involving  the  nuclei  of  the  trigeminus,  the  crossed  secondary  sensory 
paths  of  the  tegmentum,  partially  involving  the  superior  cerebellar  peduncle  (Pes) 
and  the  median  lemniscus  (Rm),  and  not  involving  the  anterior  portion  of  the  pons. 

On  the  right  there  is  hemianesthesia  of  the  extremities  of  the  syringomyeKc  type, 
above  all  for  pain  and  temperature  sense. 

On  the  left  there  is  paralysis  of  the  muscles  of  mastication  (pterygoid,  masseter, 
temporal)  by  lesion  of  the  motor  nucleus  of  the  trigeminus.  There  is  slight  anesthesia 
in  the  trigeminus  legion  (sensory  nucleus  V)  and  choreo-athetoid  movements  of  the 
extremities  from  involvement  of  the  superior  cerebellar  peduncle.  (Dejerine.)  (See 
Chart,  No.  13,  p.  392.) 


FRIEDREICH'S  ATAXIA  413 

difficulty  in  standing  and  walking.  The  patient,  in  high  grades  of 
atrophy  or  aplasia,  is  unable  to  stand,  or  sometimes  even  to  sit;  in 
the  milder  grades  the  station  is  wobbly,  the  feet  placed  far  apart,  and 
walking  is  possible  only  with  assistance.  The  gait  is  then  the  classical 
drunken  stagger.  There  is  marked  asynergia  of  the  trunk  and  lower 
extremities.  Tremors,  ataxias,  incoordination  (asynergias)  of  the 
upper  extremities  are  also  present.  Hypotonus,  muscular  weakness, 
slow,  irregular,  hesitating  or  explosive  speech  are  also  present.  Nys- 
tagmus may  or  may  not  be  present,  there  is  usually  adiadokokinesia, 
the  knee-jerks  are  usually  normal,  or  even  slightly  exaggerated  at 
times,  even  in  the  presence  of  hypotonus.  Bilateral  atrophies  show 
similar  symptoms. 

It  is  evident  that  until  the  newer  knowledge  regarding  cerebellar 
localization  is  coordinated  with  the  older  and  newer  findings  the  studies 
which  have  appeared  up  to  the  present  time  will  lack  precision. 

Combined  AjjJasias  of  the  Cerebelluvi  and  Brain. — Combette's  (old 
period)  patient,  with  absolute  absence  of  the  cerebellum  had  from  birth 
epileptiform  attacks,  was  imbecile,  had  weakness  of  the  muscles  of 
neck  and  extremities,  was  able  to  walk  but  fell  often.  Many  of  these 
patients  are  idiotic  and  imbecile,  and  show  similar  symptoms  to  those 
enumerated  in  the  previous  paragraph. 

Mingazzini  includes  the  olivo-ponto-cerebellar  atrophies  here,  but 
these  are  discussed  later. 

Holmes^  calls  these  cases  "  congenital  smallness  of  the  central  nervous 
system,  with  cerebellar  symptoms." 

A  number  of  conditions  may  be  grouped  here.  Some  of  ]\Iarie's 
so-called  hereditary  cerebellar  ataxias  are  best  referred  here.  Irregular 
staggering  gait,  Romberg,  disorder  of  speech,  nystagmus,  and  ataxias 
of  limbs  are  the  chief  symptoms.  These  patients  have  shown  small 
cerebellums  with  apparently  intact  tracts  in  cerebellum  and  cord. 

Olivo-ponto-cerebellar  Atrophy. — This  type  was  described  by  Thomas 
and  shows  a  fairly  definite  syndrome.  Anatomically  there  is  atrophy 
of  the  cerebellar  cortex,  of  the  bulbar  olive,  and  of  the  gray  matter 
of  the  pons.  There  is  total  degeneration  of  the  middle  cerebellar 
peduncles,  partial  degeneration  of  the  inferior  cellebellar  peduncles, 
and  a  relative  integrity  of  the  cerebellar  nuclei.  It  is  not  necessarily 
hereditary,  familial  nor  congenital.  It  comes  on  at  an  advanced  age 
and  progresses  slowly.  Clinically  there  is  great  defect  in  equilibration 
in  standing  and  walking,  drunken  gait.  Romberg  is  absent.  Some 
irregular  intention  tremor,  usually  nystagmus,  and  also  scanning 
speech  is  present. 

Cerebellum  and  Cord  Atrophies  or  Aplasias.^ — Here  also  a  motley 
group  is  on  record.  These  cases  will  vary  greatly  in  proportion  to 
the  varying  degree  of  the  lesion  in  the  cerebellum  and  in  the  cord. 
Marie's  hereditary  cerebellar  atrophies  belong  here.     Some  authors 

1  Brain,  1907,  p.  546.  2  Holmes,  Brain,  1907,  loc.  cit.,  for  literature. 


414 


CEREBELLAR  SYNDROMES 


Fig.  216. — Scoliosis  of  Friedreich's  ataxia. 


Fig.  217. — ^Friedreich's  ataxia,  showing  the  permanent  extension  of  the  great  toe. 


HEMORRHAGE  OF  CEREBELLUM 


415 


are  disposed  to  refer  Friedreich's  disease  to  this  group  also,  and  to 
claim  that  between  these  two  disorders  one  finds  every  gradation  from 
the  Friedreich  type,  with  mostly  cord  and  little  cerebellar  change,  to 
Marie's  type,  with  more  cerebellar  and  little  cord  change. 

The  chief  signs  here^  are  ataxias  of  the  leg,  arm,  facial,  ocular, 
head,  laryngeal,  and  pharyngeal  muscles.  Cerebellar  reeling,  no 
Romberg.  Later  palsies  and  contractions.  Exaggerated  tendon 
phenomena.  In  most  of  these  cases  the  disorder  is  predominantly  in 
the  cord. 

In  other  cases  with  degeneration  of  the  spinocerebellar  tracts  with 
normal  or  only  small  cerebellum  one  finds  patients  with  staggering 
gait,  scanning,  explosive,  slow  speech,  nystagmoid  jerkings  of  the 
eyes,  muscular  cramps,  fatigability  of  muscles,  normal  or  exaggerated 
knee-jerks.  As  mentioned,  Friedreich's  disease  properly  belongs  to  this 
rubric. 


1 

'S 

I    f 

'■*^pf 

^^Sd 

Fig.  218. — Hemorrhage  of  cerebellum.     (Larkin.) 

Primary  Parenchymatous  Degeneration. — Holmes-  has  described  this 
condition.  It  usually  sets  in  about  middle  age  and  progresses  slowly. 
Staggering  or  reeling  gait  is  an  early  symptom,  then  asynergia  of  the 
upper  extremities,  and  later  hesitating,  scanning,  or  explosive  articula- 
tion, nystagmus,  tremor  of  the  head  and  limbs.  Tendon  reflexes  are 
normal  or  slightly  exaggerated.  No  clonus,  no  Babinski.  Sphincters 
intact  and  normal  psyche.  Most  of  the  cases  have  shown  a  familial 
character. 

Hemorrhage  of  Cerebellum.^ — Cerebellar  hemorrhage  is  probably 
extremely  rare.  Its  symptomatology  will  depend  largely  on  its  size 
and  the  location  of  the  effusion.     The  superior  cerebellar  artery  is 


1  Brown,  Brain,  1892,  xv,  250. 

2  Starr,  Medical  Record,  May  2,  1906. 


2  Brain,  1907,  p.  466. 


416  CEREBELLAR  SYNDROMES 

oftenest  involved,  thus  implicating  certain  portions  of  the  dentate 
nuclei.    Extension  into  the  fourth  ventricle  is  to  be  borne  in  mind. 

The  onset  of  the  symptoms  is  usually  sudden,  or  preceded  by  pain 
in  the  back  of  the  head,  with  slight  giddiness,  or  forced  position  of 
the  head.  There  is  usually  marked  vertigo,  recurring  on  attempts 
to  move,  and  usually  persisting  in  non-fatal  cases.  Cerebellar  gait, 
forced  position  of  head,  depending  on  location,  nystagmus,  especially 
on  lateral  movements.     Operative  interference  is  generally  useless. 

Cysts  of  Cerebellum.^ — Cysts  and  cystic  tumors  are  not  always 
distinguishable.  Together  they  form  a  small  part  of  tumor  formations 
in  the  cerebellum  (5  to  10  per  cent.).  The  symptoms  of  cerebellar 
cysts  are  practically  identical  with  those  of  cerebellar  tumor,  but  the 
operative  outlook  is  much  better. 

Cerebellar  Tumors.^ — Before  discussing  the  subject  of  tumors  of  the 
cerebellum  proper  and  their  symptomatology  a  word  may  be  said 
regarding  the  question  of  cerebellar  localization.  The  researches  of 
Bolk,  and  others^  in  comparative  anatomy,  and  of  Horsley*  have 
served  to  make  fairly  certain  that  both  in  the  cortex,  and  in  the 
intrinsic  nuclei  there  are  definite  localizations,  the  former  with 
reference  to  sensory  representations  from  different  portions  of  the 
body,  the  latter  with  reference  to  motor  representations. 

With  reference  to  sensory  representation,  studying  practically  only 
the  terminations  of  Gowers'  tract,  Horsley^  concluded  that  there 
was  no  evidence  of  differentiation  of  the  cerebellar  cortex  into  localized 
receiving  stations  for  the  impressions  (muscular,  arthritic)  which 
ascend  from  the  arm,  trunk,  or  leg  muscles,  joints,  etc.,  respectively. 
This  author  holds  that  the  results  of  the  work  of  Bolk  and  others 
did  not  guard  sufficiently  against  lesions  of  the  adjacent  nuclei. 

While  this  may  be  true  for  the  distribution  of  Gowers'  tract,  it  is 
not  true  for  the  distribution  of  the  olivocerebellar  tract.  Stewart  and 
Holmes^  have  shown  that  fibers  from  certain  portions  of  the  inferior 
olive  pass  to  definite  regions  in  the  contralateral  cerebellar  cortex. 
The  function  of  these  olivocerebellar  paths  is  still  in  question.  As 
to  definite  localization  for  other  receptor  paths  (chemical,  etc.)  exact 
knowledge  is  absolutely  wanting  at  the  present  time. 

So  far  as  localization  of  motor  functions  in  the  nuclei  is  concerned, 
this  seems  to  have  received  definite  confirmation  by  the  work  of 
Horsley  {loc.  cit.). 

Cerebellar  tumors  are  relatively  frequent.  Almost  one-third  of 
the  brain  tumors  occurring  in  childhood  and  adolescence  are  of  cere- 
bellar origin.  In  order  of  frequency  one  finds  glioma,  tubercle,  sarcoma, 
and  fibroma. 

The  chief  general  symptoms  of  cerebellar  tumors  are  headache, 

1  Williamson,  Review  of  Neurology  and  Psychiatry,  March,  1910,  for  literature, 

2  Stewart  and  Holmes,  Brain,  1904,  xxvii,  522. 

3  See  Van  Rynberk,  Ergebnisse  d.  Physiol.,  1907. 

4  Brain,  1908.  sjbid.,  1909.  e  Ibid.,  1908. 


TUMORS  OF  CEREBELLUM 


417 


usually  severe,   occipital   or  frontal,   and  apt  to  be  confined  to   a 
sagittal  plane;  papilledema  and  later  optic  atrophy,  which  is  rapidly 


Fig.  219 


Fig.  220 


progressive;  vomiting,  vertigo,  and  tenderness  to  percussion  over  the 
occiput.  Tumors  in  the  hemispheres,  not  involving  the  central  tracts 
or  the  intrinsic  nuclei,  may  give  rise  to  no  localizing  symptoms.    But 

27 


418 


CEREBELLAR  SYNDROMES 


there  is  usually  an  implication  of  these  contiguous  structures  with 
added  symptoms. 

These  are  the  classical  cerebellar  syndromes  of  gait,  and  attitude, 
asynergia,  ataxia,  and  adiadokokinesia  of  the  same  side,  with  hypo- 


FiG.  221. — Tumor  of  cerebellum.      (Goodhart.) 

tonia  of  special  character  already  described,  and  motor  paresis  of  the 
same  side.    To  these  are  usually  added  nystagmus  and  eye  deviations 


Fig.  222. — "Skew  deviation"  after  removal  of  a  tumor  from  the  left  lateral  lobe 
of  the  cerebellum:  the  left  eye  is  directed  downward  and  inward,  the  right  eye  upward 
and  outward.     (Holmes  and  Stewart.) 


already  noted.  The  nystagmus  is  apt  to  be  pronounced  only  on 
looking  to  the  affected  side,  and  is  usually  slower  and  coarser  than  the 
nystagmus  of  labyrinthine  origin,  or  of  involvement  of  the  vestibular 
tracts.    As  these  latter  are  frequently  impinged  upon,  it  is  useless  to 


CEREBELLAR  ABSCESS  419 

insist  upon  too  fine  distinctions  in  the  character  of  nystagmus.  Unless 
the  pyramidal  tracts  are  influenced  by  pressure,  the  arm  and  leg  tendon 
reflexes  are  not  markedly  exaggerated,  nor  are  they  lost,  and  the 
Babinski,  Oppenheim,  Schaefer  and  Remak  signs  of  pyramidal  tract 
involvement  are  not  present.  The  abdominal  reflexes  are  usually 
unmodified. 

As  the  tumors  increase  in  size  there  are  added  symptoms  due  to 
encroachments  or  pressure  upon  contiguous  structures.  These  are 
usually  the  signs  of  involvement  of  the  pyramidal  tracts,  eye  palsies, 
and  of  the  cranial  nerves — from  the  fifth  to  the  twelfth.  These  all 
show  on  the  opposite  side  of  the  body.  The  two  lower  branches  of 
the  facial  are  involved,  the  tongue  protrudes  to  the  paralyzed  side, 
and  is  without  atrophy  or  R.  D.  Occasionally  the  medulla  is  pressed 
upon  and  one  finds  all  branches  of  the  facial  involved  with  atrophy  of 
the  tongue.  Homolateral  anesthesia  of  the  cornea  may  be  present, 
due  to  trigeminus  involvement.  Homolateral  affections  of  the  ears, 
deafness,  buzzing  and  homolateral  pain  to  pressure  on  the  mastoid 
may  aid  in  diagnosis.  Percussion  should  never  be  neglected.  Oppen- 
heim has  called  attention  to  the  cracked-pot  sound  often  present  in 
cerebellar  tumors. 

Unilateral  signs  are  apt  to  pass  over  into  bilateral  signs  as  the  press- 
ure increases,  with  dysarthria,  dysphagia,  continued  vomiting,  and 
finally  cardiac  and  respiratory  signs. 

Lumbar  puncture  may  give  important  information  in  clearing  up 
a  diagnosis  of  meningitis-serosa  or  hydrocephalus. 

Cerebellar  Abscess. — These  are  relatively  frequent,  and  originate 
mainly  from  middle-ear  infections,  either  by  way  of  the  temporal 
lobes  or  the  mastoid,  and  wounds,  from  trauma,  which  latter  may 
have  occurred  a  long  time  previously.  Occasionally  abscess  may 
result  from  thrombi  due  to  abscess  of  the  lungs,  ulcerative  endocar- 
ditis, etc. 

These  abscesses  vary  considerably  in  size  from  that  of  a  pea  to  an 
apple,  and  their  development  is  either  acute  or  chronic. 

The  characteristic  symptoms  are  headache,  usually  occipital,  and 
radiating  into  the  neck  region,  producing  marked  stiffness  of  the 
neck,  at  times  resembling  the  pain  of  a  cerebrospinal  meningitis. 
General  unrest,  nausea,  vomiting  and  stupor  are  present.  Tem- 
perature may  be  added,  but  a  cerebellar  abscess  may  run  a  course  of 
several  months  without  temperature.  Of  special  cerebellar  symptoms 
ataxia,  nystagmus  and  rotatory  vertigo  are  characteristic.  This 
vertigo  is  usually  rendered  worse  and  vomiting  is  induced  by  move- 
ment of  the  body;  hemiparesis  and  hemiasynergia  are  usually  present 
on  the  side  of  the  lesion,  but  this  is  not  a  constant  sign. 

By  an  increase  in  the  size  of  the  abscess,  symptoms  of  pressure, 
precisely  similar  to  those  mentioned  under  Tmiior,  may  develop. 
Papilledema,  with  optic  atrophy,  is  not  infrequent  in  large  abscesses. 

Brain  puncture  by  special  aspiration  needles  is  advisable  to  clear 


420  CEREBELLAR  SYNDROMES 

up  the  diagnosis  in  complicated  cases.  Lumbar  puncture  is  useful  in 
excluding  meningitis. 

Associated  Posterior  Fossa  Complications. — Occipital  Lobes. — These 
may  be  pressed  upon  anterosuperiorly  by  a  foreign  body  growing  upon 
or  within  the  superior  lobe  of  the  cerebellum.  Hemianopsia  (mind 
blindness)  may  then  occur. 

Corpora  Quadrigemina. — Ocular  palsies  of  a  nuclear  character  occur, 
first  on  one  side,  then  upon  the  other.  The  oculomotor  and  abducens 
are  oftener  involved  than  the  trochlear.  There  is  less  apt  to  be  a 
paralysis  of  accommodation  or  changes  in  the  pupils,  and  the  loss  of 
conjugate  action  of  the  eye  is  rarely  found.  Implication  of  the  pos- 
terior corpus,  and  of  the  middle  geniculate  causes  deafness,  one-  or 
both-sided,  and  if  the  lateral  geniculate  be  pressed  upon,  amblyopia, 
without  papilledema.    Pineal  tumors  may  cause  the  same  picture. 

Cerebral  Peduncles. — If  these  structures  are  markedly  impinged 
upon  the  Weber-Gubler  syndrome  of  alternate  hemiplegia  and  oculo- 
motor, palsy  may  be  found.  From  milder  irritative  pressures  one 
obtains  the  Benedict  syndrome,  oculomotor  palsy,  with  tremor  of  the 
opposite  side.  If  the  lemniscus  is  impaired  there  is  crossed  anesthesia 
and  ataxia.     (See  Midbrain  Syndromes.) 

Pons. — Here  one  finds  a  number  of  syndromes  added  to  the  initial 
cerebellar  symptoms.  Crossed  hemiplegia  with  facial  palsy  (Millard- 
Grubler)  and  R.  D.  Occasionally  from  a  more  anterior  pressure 
there  may  be  homolateral  facial  palsy  without  R.  D. 

Crossed  Hemiplegia  and  Abducens  Palsy. — Both  are  usually  asso- 
ciated with  hypoglossal  involvements.  Conjugate  deviations  to  the 
opposite  side  of  the  lesion  is  observed  in  these  cases  when  the  abducens 
nucleus  is  involved.  Such  conjugate  palsies  speak  more  for  tumors 
within  the  pions  {q.  v.).    (See  Midbrain  Syndromes.) 

Crossed  hemiplegia  and  trigeminus  involvement  is  occasionally 
found,  and  also  alternate  hemiplegias  with  cochlearis  symptoms. 
Here  the  hearing  defect  is  due  to  destruction  of  the  intrapontine 
fibers  of  the  cochlearis;  or  to  pressure  on  the  tuberculum  acousticum. 

Tumors  of  the  Fourth  Ventricle. — These  may  be  considered  here 
because  of  the  cerebellar  symptoms  induced.  These  tumors  are  for 
the  most  part  glioma,  sarcoma,  psammoma,  carcinoma.  They  give 
rise  to  symptoms  due  to  pressure  on  the  medulla  and  pons  and  almost 
always  cause  a  marked  hydrocephalus  with  advancing  stupor  and 
confusion.  Cysticercus  may  also  be  found  here.  Bruns  has  called 
attention  to  the  following  features.  Alternating  periodicity  of  head- 
ache, nausea,  vomiting,  vertigo,  changes  in  pulse  and  breathing, 
with  sudden  let  up  of  all  these  symptoms.  The  vertigo  and  vomiting 
are  set  up  by  changes  in  position  of  the  head;  sudden  movement  of 
the  head  may  cause  immediate  unconsciousness.  Cerebellar  ataxia, 
mild  nystagmus  and  occasionally  diplopia  are  other  signs. 

One  more  word  may  be  said  about  cerebellar  tumors  and  their 
diagnosis.    They  may,  in  the  presence  of  few  signs  only,  be  mistaken 


CEREBELLOPONTINE  ANGLE   TUMORS 


421 


for  affections  of  the  frontal  lobes  (frontocerebellar  paths),  parietal 
lobes  (implications  of  central  sensory  components  and  of  the  optic 
thalamus) . 

In  frontal  lobe  tumors  special  intelligence  defects  are  usually  found, 
if  carefully  examined  for  by  the  methods  of  Ziehen,  Sommer,  and 
Kraepelin.  The  tremor  is  apt  to  be  very  fine  and  rapid,  hemiparesis, 
if  present,  is  crossed,  and  shows  spastic  phenomena;  the  speech  dis- 
turbance is  aphemic;  the  conjugate  deviations  are  irritative  and  not 
paralytic.  Then  anosmia,  apraxia,  and  aphasia  are  often  added.  Skew 
deviations,  and  hypotonus  are  not  known  for  frontal  tumors. 


Fig.  223. — Tumor  (neurofibroma)  of  cerebellopontine  angle.      (Larkin.) 


Parietal  lobe  disease  only  occasionally  offers  difficulties,  while  the 
characteristic  sensory  disturbances  and  central  pains  of  optic  thalamus 
involvement  should  exclude  this  structure. 

Cerebellopontine  Angle  Tumors.^- — These  should  be  discussed  here 
because  of  the  symptoms  of  cerebellar  pressure  and  of  vestibular 

'  Henschen,  F.,  Ueber  Geschwiilste  der  liinteren  Schadelgrube  imbescndere  des  Klein- 
hirnbrlickenwinkels,  1911,  for  full  literature  to  date;  also  English  literature,  Fraenkel 
and  Hunt,  Medical  Record,  1903,  and  Medical  News,  1904;  Stewart  and  Holmes,  Brain, 
1904;  Weisenberg,  Jour.  Amer.  Med.  Assoc,  1908;  Starr,  Jour.  Nerv.  and  Ment.  Dis., 
1910;   Lewandowsky,  Handbuch  der  Neurologic. 


422  CEREBELLAR  SYNDROMES 

involvement.  Two  main  types  of  tumor  come  under  review.  Those 
from  the  pia  of  the  cerebellum,  and  those  growing  on  or  about  the 
eighth  nerve.  Fibromata,  m.yomata,  and  sarcomata  are  the  most 
frequent. 

These  tumors  press  upon  the  pons  and  middle  cerebellar  peduncle 
and  the  symptoms  vary  slightly,  according  to  the  variations  in  press- 
ure on  these  two  structures.  The  eighth  nerve  is  usually  involved 
early;  buzzing  and  deafness  are  observed.  Facial  palsy  is  usual  with 
corneal  anesthesia  from  pressure  on  the  fifth;  trigeminal  pains  are 
frequent.  Ptosis  may  come  on.  Pressure  on  the  cerebellum  causes 
the  typical  gait  and  the  ataxia,  homolateral  paresis,  and  hypotonus. 
A  contralateral  paresis  from  pressure  on  the  pyramidal  tracts  usually 
develops.  This  gives  the  usual  signs  of  a  pyramidal  tract  involvement. 
Homolateral  static  tremor  is  frequent,  and  a  sense  of  subjective  rotation 
toward  the  side  of  the  lesion. 

Treatment  of  Cerebellar  Conditions. — Gummata  must  be  attacked  by 
the  usual  antisyphilitic  treatment,  otherwise  surgery  offers  the  only 
opportunity,  and  this  is  limited  to  the  attack  upon  cysts  which  is 
apt  to  give  fortunate  results.  The  results  of  operations  for  abscess 
are  improving,  as  are  also  those  upon  tumors.  But  as  each  case  is 
a  law  unto  itself,  it  is  futile  to  generalize. 


CHAPTER  X. 

PARALYSIS  AGITANS,  CHOREA,  AND  RELATED 
DISORDERS. 

PARALYSIS   AGITANS. 

History. — The  clinicians  of  the  times  immediately  preceding  Parkin- 
son grouped  the  paralysis  agitans  cases  of  the  present  day  in  several 
different  categories.  Galen  had  noted  the  characteristic  tremor,  and 
the  class  of  tremors  of  this  kind;  the  "tremblement  palpitant"  of 
Preysinger  was  part  of  the  earlier  palmos  of  Galen.  Francis  de  la 
Boe  was  shrewd  enough  to  notice  the  difference,  afterward  forgotten, 
between  the  tremor  produced  by  attempts  at  motion  and  the  tremors 
present  while  the  limbs  were  at  rest,  and  his  term  tremor  coactus, 
for  the  tremors  of  paralysis  agitans,  was  utilized  up  to  Parkinson's 
time.  Juncker  had  also  described  a  tremor,  a  paralytic-like  tremor, 
tremores  paralytoidei,  which  included  some  of  these  patients. 

Not  only  was  the  tremor  appreciated,  but  the  clinicians  of  the 
eighteenth  century  (Gaubius,  1751),  had  called  attention  to  the  pro- 
pulsion of  these  patients,  and  Sauvages  groups  them  in  his  choreas, 
as  Scelotrybe  precipitee  (Danse  de  St.  Guy  precipitee,  L.). 

Parkinson,  in  his  famous  thesis  on  the  "Shaking  Palsy,"  London, 
1817,  made  a  synthesis  of  several  of  these  conditions,  and  erected  a 
new  clinical  form  to  which  he  gave  the  name  shaking  palsy  (paralysis 
agitans),  and  gave  the  following  short  and  striking  description: 
"Involuntary  tremulous  motion,  with  lessened  muscular  power,  with 
a  propensity  to  bend  the  trunk  forward,  and  to  pass  from  a  walking 
to  a  running  pace,  the  senses  and  the  intellects  being  unimpaired." 

All  of  the  case  histories  cited  by  Parkinson  were  probably  true 
cases  of  our  present-day  paralysis  agitans,  but  the  group  as  then 
understood  still  contained  certain  of  the  chronic  choreas,  and  certain 
cases  of  multiple  sclerosis,  possibly  certain  thalamic  cases,  etc.,  which 
later  clinicians  have  come  to  recognize.  The  chronic  choreas  were 
definitely  excluded  by  the  work  of  the  Germain  See,  1851,  and  the 
researches  of  B.  Cohn,^  Ordenstein,^  and  Charcot  finally  separated  the 
multiple  sclerosis  syndrome. 

Since  the  appearance  of  Charcot's  studies  the  monographs  of 
Wollenberg,  Heimann,  Zingerle,  1910,  and  of  Mendel,  1911,  contain 
the  chief  steps  made,  showing  the  steadily  advancing  trend  to  regard 

1  Wien.  med.  Woch.,  No.  18. 

2  TMse  de  Paris,  1868. 


424     PARALYSIS  AGITANS,   CHOREA,  RELATED  DISORDERS 

the  disorder  not  in  the  Hght  of  a  functional  disturbance,  as  Charcot 
taught,  but  as  an  organic  syndrome,  and  affecting  either  circum- 
scribedly  or  more  diffusely,  certain  cerebellar  and  thalamic  mechanisms. 

Etiology. — The  changes  of  age  seem  to  be  the  most  striking  etio- 
logical factors.  The  majority  of  the  patients  are  between  50  and  70 — 
although  cases  of  patients  of  19,  15,  12,  10,  and  3  years  of  age  are 
recorded,  some  of  which  have  been  possibly  faultily  diagnosed,  the 
vast  majority  of  them  being  multiple  sclerosis  or  encephalitis.  Hered- 
itary factors  may  play  a  role,  probably  through  vascular  disease. 
Berger,  Gowers,  Borgherini,  Clerici  and  Medea  and  others  have 
reported  cases  occurring  in  two  generations  or  in  more  than  one 
member  of  the  same  generation,  and  Erb  reports  that  in  15  per  cent, 
of  his  cases  the  parents  or  grandparents  suffered  from  the  same  disease. 
Concerning  indirect  heredity,  the  least  said  the  better,  as  the  studies 
available  are  entirely  too  conflicting,  and  for  the  most  part  inapplicable. 

Emotional  disturbances  are  held  accountable  by  many  writers;  it 
is  difficult  to  determine  here  whether  one  is  concerned  with  cause  or 
effect.  Sorrow,  worry  and  emotional  distress  are  such  universal  all- 
pervading,  environmental  factors,  that  too  much  stress  must  not  be 
placed  upon  them.  Sudden  shock  may  perhaps  stand  in  an  accen- 
tuating, accidental  relationship. 

Trauma  stands  in  a  possibly  closer  relationship.  It  is  highly  improb- 
able as  a  direct  cause,  but  it  may  be  a  sufficiently  exciting  cause  to 
bring  the  symptoms  of  a  slumbering  paralysis  agitans  to  the  surface, 
or  those  of  a  mild  case  rapidly  to  a  severe  stage. 

Physical  stress  is  a  factor  which,  bearing  upon  arteriosclerosis,  may 
be  an  accompanying  factor  in  certain  cases.  Toxic  factors  of  them- 
selves are  not  known  to  play  any  necessary  role.  Their  coincidental 
occurrence  is  frequently  reported ;  the  same  may  be  said  of  infections. 
Cold,  exposure  to  wet,  and  other  factors  are  probably  more  accidental 
than  vital;  they  may  augment  the  action  of  an  underlying  factor,  as 
yet  unknown;  they  may  represent  purely  coincidental  features. 

Arteriosclerosis  is  the  chief  factor  in  bringing  about  the  syndrome. 
The  central  features  of  the  syndrome  are  matters  of  localization  in 
the  implication  of  certain  pathways  by  the  sclerosing  process. 

The  parathyroid  hypothesis  is  unproved. 

Symptoms. — These  develop  for  the  most  part  very  slowly,  although 
occasionally  patients  are  seen  who  show  fulminating  types,  and 
although  atypical  developments  are  known,  the  regularity  and 
uniformity  in  the  development  is  very  striking. 

The  vast  majority  of  the  patients  show,  on  close  analysis,  prodromal 
symptoms  which  are  chiefly  sensory,  in  contrast  to  the  sensorimotor 
symptoms  of  the  more  advanced  stages  of  the  picture.  The  more 
characteristic  of  these  prodromata  are  fugitive,  irregular  pains  of  a 
sharp,  lancinating  character,  frequently  found  in  the  extremities  first 
to  be  affected  by  the  motor  disturbances,  and  usually  ceasing  as  these 
latter  advance.     Paresthesise  are  also  frequent,   causing  sensations 


PARALYSIS  AGITANS  425 

of  tickling,  cold  spots,  hot  spots,  gastric  distress— almost  crises-like 
attacks,  with  diarrhea  and  colicky  disturbances  in  the  large 
intestine. 

General  malaise  with  headache,  sweating,  mild  vertigo,  palpitation, 
sialorrhea,  anxiety,  pressure  of  blood  in  the  head,  easy  excitability, 
these  are  general  symptoms  accompanying  many  senile  and  presenile 
conditions,  but  are  so  frequently  found  as  forerunners  of  the  motor 
symptoms,  and  persist  with  such  marked  increase  of  severity  through- 
out the  disorder  that  their  appearance  is  to  be  regarded  as  more  than 
coincidental. 

The  symptoms  of  the  more  classical  syndrome  may  be  grouped  as 
follows:  (1)  The  main  group  of  sensorimotor  disturbances,  varying 
in  intensity  and  location  in  different  individuals.  (2)  A  number  of 
sensory,  vasomotor,  trophic  and  secretory  disturbances  already  indi- 
cated as  often  in  part  occurring  as  prodromes.  (3)  Psychical  symptoms 
which  are  somewhat  variable  and  possibly  not  essentially  related  to 
the  disorder  per  se. 

The  sensorimotor  disturbances  are  predominantly  increase  of  mus- 
cular tonus,  with  rigidity  and  resulting  contractures,  and  motor  dis- 
turbances with  tremor,  compulsory  gait,  forced  attitudes,  forced 
movements,  and   loss   of  mimetic  expression. 

An  increase  in  the  muscular  tonus  is  a  most  fundamental  feature 
in  the  concept  paralysis  agitans.  As  a  result  of  it  there  follows 
the  rigidity,  the  mask-like  countenance,  and  the  contractures.  The 
increase  in  the  muscle  tonus  usually  is  a  very  early  sign,  although 
positive  traces  of  rigidity  may  not  appear  until  later.  It  is  practically 
always  found,  whereas  there  are  some  patients  who  have  little  or  no 
tremor^  and  yet  the  name  paralysis  agitans  is  properly  used.  Asso- 
ciated with  the  hypertonus  and  the  rigidity  there  is  a  slowness  of 
movement,  and  a  steadily  increasing  stiffness,  and  also  retardation 
of  the  motor  and  ideational  impulses. 

The  muscular  rigidity  varies  widely  in  its  situation  at  the  beginning. 
Practically  the  symptoms  first  become  manifest  on  one  side  of  the 
body,  and  the  severity  of  the  symptoms  usually  predominates  on  one 
side,  it  may  be  for  years.  In  the  well-developed  syndrome  the  rigidity 
effects  particularly  the  muscles  of  the  neck  and  trunk;  the  patient 
assumes  the  bent-over  attitude,  such  as  one  naturally  assumes  when 
shivering  from  the  cold  and  the  face  is  mask-like  (corrugators)  and 
staring,  the  eye  muscles  also  sharing  in  the  rigidity  with  Stelwag's 
sign. 

Whereas  the  muscles  of  the  neck  and  back  are  most  affected,  almost 
any  group  of  muscles  may  be  involved.  The  arms  and  legs  are  almost 
always  implicated,  and  so  are  also  the  muscles  of  the  face.  Occa- 
sionally there  is  ptosis,  or  the  patients  open  their  eyes  after  closing 
them  with  difficulty.    One  of  us  has  seen  this  as  an  initial  symptom. 

1  Furstner  et  al.;  see  Zingerle. 


426     PARALYSIS  AGITANS,  CHOREA,  RELATED  DISORDERS 

The  patients  read  with  difficulty  because  of  the  stiffness  of  the 
ocular  movements.  Ocular  palsies  may  result — pseudo-ophthalmo- 
plegias.  One  occasionally  finds  slow  pupillary  reactions.  The  pharyn- 
geal and  laryngeal  muscles  being  involved,  as  others  in  the  body,  results 
in  slow,  difficult  speech,  becoming  fainter  and  fainter  as  the  years  go 
by,  until  finally  the  patient,  in  addition  to  being  unable  to  move,  to 
dress  himself,  eat  without  help,  finally  is  unable  to  talk  or  to  swallow. 
This  hypertonus  and  rigidity,  however,  is  not  associated  with  the 
usual  increased  reflex  signs  of  pyramidal  tract  involvement,  the 
reflexes  are  either  normal,  or  only  slightly  exaggerated,  no  clonus, 
Babinski,  Oppenheim,  etc.,  and  the  contractures  may  be  easily  over- 
come by  passive  movements,  in  marked  contrast  to  the  contractures 


Fig.  224. — Attitude  of  paralysis  agitans  patient.     (T-ilney.) 

of  psychomotor  cortical  origin.  The  muscular  power  is  also  not  so 
involved,  the  patients  show  muscular  weakness,  but  not  paralysis. 
There  is  a  striking  contrast  between  the  strength  of  active  movements 
and  that  of  resistance  movements.  The  former  are  weak,  the  latter 
rarely  less  than  normal. 

A  few  cases  of  extensor  rigidity  are  recorded,  but  it  is  predominantly 
of  the  flexor  muscles. 

With  the  rigidity  there  is  a  feeling  of  tension  that  the  patients  dis- 
like usually  much  more  than  the  almost  universal  tremor.  This 
causes  them  to  feel  as  though  they  were  bound.  Their  motor  impulse 
seems  interfered  with.  This  may  even  affect  their  urination  and 
defecation,  and  their  deglutition. 


PARALYSIS  AGITANS  427 

Attitude. — This  is  that  of  a  decerebellate  rigidity  in  contrast  to  a 
decerebrate  rigidity. 

Tremor. — In  the  majority  of  the  cases  this  objective  sign  seems  to  be 
the  first,  although,  in  reahty,  vasomotor  signs  precede  as  a  rule.  It 
was  the  symptom  first  noted  by  Galen.  It  is  characterized  by  its 
uniformity  and  steadily  increasing  severity,  both  in  point  of  advance 
of  the  disease,  and  also  during  the  movements  themselves.  It  is  a 
tremor  that  Franciscus  Sylvius  first  noted  was  present  while  the 
limb  was  at  rest.  It  ceases  during  movement,  especially  if  the  move- 
ment is  rapid,  and  in  the  beginning  of  the  disease.  In  the  later  stages 
it  becomes  continuous,  and  some  patients  with  paralysis  agitans  show 
some  intention  tremor.  Again,  a  certain  number  of  cases  show  little 
or  no  tremor. 

The  tremor  is  characterized  by  the  uniformity  of  its  excursions, 
which  are  at  first  small,  slow,  and  rhythmical.  They  average  about 
three  to  five  in  a  second,  according  to  the  muscles  involved.  Tremor 
in  the  muscle  of  the  thumb,  which  is  often  an  early  sign,  gives  rise  to 
the  well-known  "pill-rolling"  movements.  Similarly,  one  has  the 
movement  of  "beating  the  drum,"  and  other  conditions  when  the 
larger  muscles  of  the  arms  are  implicated.  The  muscles  involved  in 
the  early  tremors  vary  considerably;  usually  the  upper  extremity  is 
involved  before  the  lower,  the  hand  particularly;  but  with  the  prog- 
ress of  the  disease  the  tremor  tends  to  become  widespread,  almost 
universal;  most  diverse  localizations  are  on  record,  anything  is  to  be 
expected.  There  is  no  great  preponderance  of  one  hand  over  the  other, 
although  there  is  a  marked  tendency  to  disproportion  in  the  severity, 
and  a  hemiplegic  type  of  onset  and  persistence  is  frequent,  if  not 
characteristic.     Monoplegic  types  are  encountered. 

At  first  the  tremor  is  absent  during  sleep,  but  in  the  advanced 
stages  it  frequently  persists  and  constitutes  one  of  the  factors  in 
sleeplessness  which  finally  exhausts  the  patient. 

Motion  tends  to  diminish  the  tremor,  as  also  does  attention;  emo- 
tional disturbances  and  cold  increase  it  markedly.  Grasping  the 
tremulous  member,  touching  it,  or  changing  its  position  results  in  a 
temporary  cessation  of  the  tremor.  The  restlessness  of  these  patients 
is  largely  dependent  upon  the  constant  and  continual  shifting  of  the 
body,  i.  e.,  before  they  become  too  rigid,  in  order  to  obtain  comfort. 

Statistical  studies  show  that  tremor  may  be  absent  in  as  high  as 
20  per  cent,  of  the  cases — a  much  greater  proportion  than  the  rigidity 
which  also  may  be  absent  or  scarcely  noticeable.  These  are  variations 
in  precise  pathway  blocking,  the  lesions  showing  slight  variability  in 
localization. 

Disturbances  of  Equilibrium. — Propulsion,  which  apparently  was 
first  noted  by  Gaubius  (1751),  is  one  of  the  cardinal  symptoms  in 
Parkinson's  original  definition.  The  patient  on  walking  tends  to  fall 
forward,  and  in  his  efi^ort  to  keep  his  equilibrium  goes  faster  and  faster, 
until  he  either  falls  or  stops  himself  by  a  stick  or  a  foreign  body. 


428     PARALYSIS  AGITANS,   CHOREA,  RELATED  DISORDERS 

Lateropulsion  and  retropulsion  are  also  present.  These  are  due  either 
to  the  stiffness  with  the  slowness  of  muscular  movement,  or  to  a  central 
disturbance  of  equilibrium,  which  latter  is  perhaps  the  preferable 
explanation,  since  exquisite  examples  of  gait  disturbances  are  known 
without  any  marked  stiffness  or  rigidity.  In  a  few  cases  the  loss  of 
equilibrium  in  one  direction  alters  that  of  another  during  the  course 
of  the  disease. 

Secretory,  Vasomotor,  Trophic  Disturbances. — These  make  up  the 
second  category  of  symptoms  almost  universally  found  to  a  greater 
or  less  degree  in  paralysis  agitans.  As  noted,  many  are  prodromal 
symptoms.  What  relation  these  symptoms,  all  of  which  have  some 
definite  relation  to  the  sympathetic  nervous  structures,  possibly  at 
lenticular  levels,  bear  to  the  almost  universally  present  arterio- 
sclerosis is  not  yet  apparent. 

The  most  important  of  the  secretory  changes  are  increased  perspira- 
tion— sometimes  unilateral,  increased  salivation — one  of  the  most 
distressing  of  the  symptoms — and  polyuria,  with  occasionally  diarrhea. 

Among  the  vasomotor  changes  are  rushes  of  blood  to  the  head, 
reddening  of  the  face,  cyanosis,  tachycardia,  acroparesthesia,  with 
hot  and  cold  spots,  alteration  of  temperature — sometimes  unilaterally 
disposed,  and  dermographia,  which  is  almost  constant. 

Trophic  changes  in  the  skin,  such  as  atrophy,  thickening,  edema,  are 
among  the  rarer  findings. 

Psychic  Disturbances.- — These  probably  do  not  constitute  an  essential 
part  of  the  disorder,  but  represent  almost  normal  psychological 
reactions  to  a  most  distressing  and  hopeless  situation.  Depression, 
anxiety,  ideas  of  self-destruction,  scornfulness,  savage  raillery,  sarcastic 
pessimism,  euphoric  compensation  and  sublimation,  resignation  to  the 
will  of  God,  etc.,  these  are  but  a  few  of  the  innumerable  attitudes  which 
these  patients  show  at  one  or  another  time  during  their  long  period  of 
almost  unbearable  suffering. 

Parkinson  said  the  "senses  and  the  intellects  are  not  impaired," 
and  intelligence  tests  bear  this  out.  In  some  patients  one  naturally 
finds  a  senile  dementia,  and  for  most,  in  the  later  stages,  the  mental 
signs  of  an  arteriosclerotic  dementing  process  are  present.  Acute 
exhaustion,  delirious  states,  often  close  the  sad  chapter;  but  these 
are  not  a  part  of  the  paralysis  agitans. 

Sensory  Symptoms. — Tactile,  thermal  or  pain  disturbances  are  not 
definite.  They  do  not  constitute  striking  features  of  the  disorder. 
The  early  pains  are  usually  fugitive,  and  apart  from  the  dull  and  most 
oppressive  sensation  due  to  the  tension  and  stiffness,  pain  is  not 
prominent.  Irregular  anesthesiee,  hyperesthesise,  paresthesise  are 
frequently  found,  but  are  so  inconstant  that  one  can  say  that  definite 
sensory  disturbances  do  not  belong  to  the  paralysis  agitans  picture. 
When  present  in  striking  fashion  they  are  due  to  some  complicating 
factors. 

The  reflexes  are  not  markedly  disturbed.     Considerable  variation 


PARALYSIS  AGITANS  429 

exists,  but  there  is  no  constant  picture  as  yet  known  which  is  pathog- 
nomonic of  the  condition.  Reflex  activities  due  to  pyramidal  tract 
involvement  are  occasionally  found.  Increased  knee-jerks,  clonus,  and 
Babinski  phenomena  are  at  times  found,  but  they  are  not  constant, 
and  represent  occasional  rather  than  essential  features. 

Loss  of  the  Achilles  reflex  is  a  not  infrequent  symptom,  the  signifi- 
cance of  which  is  as  yet  not  definitely  placed. 

The  abdominal,  cremasteric,  epigastric,  and  anal  reflexes  are  not 
involved. 

Other  clinical  findings  are  inconstant.  The  blood  practically  shows 
nothing,  some  anemia  at  times,  but  nothing  striking;  the  cerebro- 
spinal fluid  is  practically  negative.  The  urine,  apart  from  a  frequent 
polyuria,  shows  no  quantitative  or  qualitative  anomalies,  beyond  the 
excessive  phosphate  elimination,  which  is  indicative  of  the  exhaustion. 

Course  and  Progress, — Rudimentary  forms  are  not  unknown.  Many 
senile  patients  show  conditions  closely  approaching  the  milder  grades 
of  paralysis  agitans,  and  intermediary  stages,  with  muscular  stiffness, 
slowness  of  movement,  retardation  of  motor  impulses,  etc.,  are  not 
infrequent.  Certain  stationary  cases,  non-progressing  for  twenty-five 
years,  are  also  known,  and  infrequently  patients  make  partial  recov- 
eries. These  are  possibly  syphilitic  cases  showing  the  syndrome. 
But  the  usual  course  of  the  more  frequent  arteriosclerotic  cases  is  a 
long,  slow,  and  gradual  progression,  lasting  over  many  years  with 
annoyance,  inconvenience,  discomfort,  distress,  and  agony  until  life 
becomes  a  burden.  Remissions  and  exacerbations  belong  to  almost 
every  case. 

Patients  with  little  hypertonus  and  muscular  rigidity  seem  to  pro- 
gress less  rapidly,  and  many  cases  beginning  in  younger  individuals 
do  fairly  well.  Emotional  disturbances  act  badly,  almost  invariably 
causing  marked  progress  of  the  disease. 

The  majority  of  the  patients  get  worse  gradually.  The  increasing 
weakness  and  stiffness  limits  them  more  and  more  in  their  work,  until 
that  becomes  impossible.  Then  the  walking,  to  which  they  are  un- 
consciously attracted,  becomes  more  and  more  difficult.  They  are  then 
confined  to  their  chairs  for  a  few  years,  or  to  bed,  and  finally  come  to 
be  helpless  invalids,  who  must  be  fed,  turned  over,  attended  to  like 
living  rigid  statues,  which  in  the  presence  of  relatively  intact  intelli- 
gence, though  robbed  of  the  power  of  expression,  reading,  writing, 
even  pantomime,  constitutes  one  of  the  most  ghastly  afilictions  in  the 
entire  realm  of  nervous  disorders. 

Decubitus,  pneumonia,  exhaustion  delirium,  and  starvation,  are  the 
usual  precursors  to  the  end  of  a  disorder  whose  prognosis  is  bad. 
Recoveries  there  are  none,  although  stationary  cases  are  occasionally 
seen. 

Differential  Diagnosis. — The  diagnosis  is  rarely  difficult.  The  atti- 
tude, gait,  facial  expression,  and  tremor  are  so  characteristic  as  to 
stamp  the  patient  at  once.     In  the  initial  period,  before  the  stiffness, 


430     PARALYSIS  AGITANS,   CHOREA,   RELATED  DISORDERS 

tremor,  pulsions,  etc.,  have  developed,  the  diagnosis  may  be  difficult, 
but  after  its  classical  development  it  cannot  be  mistaken  for  anything 
else. 

Hysteria  sometimes  comes  into  review,  but  here  the  character  of 
the  hypertonus  is  quite  different,  the  tremor  is  rarely  classical,  and 
can  be  more  readily  influenced  by  diversion  and  distraction.  The 
exaggeration  of  the  paralysis  agitans  symptom-picture  is  characteristic 
of  the  hysterical  type. 

Multiple  sclerosis  of  the  cerebellar  type  frequently  shows  the  classi- 
cal paralysis  agitans  picture,  plus  the  evidence  of  pyramidal  tract 
involvement,  nystagmus,  bulbar  speech,  etc.,  of  this  disease.  It  is 
usually  present  in  younger  individuals.  Most  of  the  so-called  juvenile 
Parkinson  cases  are  cerebellar  types  of  multiple  sclerosis. 

Senile  and  presenile  tremors  have  been  mentioned. 

Complicating  diseases,  such  as  tabes,  hysteria,  multiple  sclerosis, 
hemiplegia,  exophthalmic  goitre,  etc.,  are  known. 

Pathology. — ^A  dogmatic  presentation  of  the  causes  for  paralysis 
agitans  is  not  yet  available.  The  trend  of  opinion  is  that  it  represents 
a  senile  or  presenile  degeneration  of  certain  brain  regions,  and  these 
are  mostly  contained  within  the  cerebellar,  thalamic,  and  lenticular 
mechanisms.  Whether  they  are  confined  to  the  midbrain  regions  is 
not  proved — neither  are  these  mechanisms — but  the  evidence  points 
in  that  direction.  The  increased  tonus  resembles  cerebellar  and  not 
cerebral  tonus.  The  rigidity,  attitude,  slowness  of  motor  impulses, 
has  its  analogies  in  disorders  of  the  frontocerebellar,  cerebello-rubro- 
spinal  and  thalamic  systems.  The  disturbances  of  equilibrium  are 
distinctly  of  the  cerebellar  type.  The  vasomotor,  secretory,  and 
trophic  symptoms  represent  central  sympathetic  disorders  which  are 
referred  with  greatest  probability  to  those  thalamic  nuclei  other  than 
the  nuclei  which  are  known  to  be  the  synapses  of  the  chief  sensory 
pathways. 

The  recent  review  of  Zingerle  (loc.  cit.)  brings  these  features  to  the 
fore.  Few  complete  series  of  microscopic  sections  through  the  cere- 
bellum and  midbrain  region  have  as  yet  been  studied,  but  distinct 
atrophies  in  the  ansa  lenticularis,  in  thalamic,  and  lenticular  regions 
are  present  in  those  studied  by  the  serial  section  method. 

The  muscular  hypotheses,^  which  are  many,  are  totally  inadequate, 
as  is  also  the  parathyroid  hypersecretion  theory  upheld  by  Lundborg, 

As  to  the  nature  of  the  process  that  brings  about  the  degenerations 
in  the  regions  involved,  science  is  still  in  the  dark.  Arteriosclerosis 
is  probably  the  chief  factor. 

Therapy. — Notwithstanding  the  gloomy  outlook,  much  can  be  done 
to  relieve  the  patients.  They  must  be  guarded  against  cold,  and  as 
far  as  possible,  from  emotional  disturbances  and  mental  and  physical 
strain. 

1  Camp,  Jour.  Amer.  Med.  Assoc,  1910. 


DYSTONIA   MUSCULORUM  DEFORMANS  431 

They  should  Hve  in  warm,  dry,  sunny  rooms  if  possible,  be  much 
in  the  open  air,  eat  a  full,  mixed  diet,  and  possibly  a  minimum  of 
purin-containing  substances  is  needed.  Alcohol  and  coffee  may  be 
used  in  moderation.  Tobacco  is  not  necessarily  taboo;  two  to  three 
cigarettes  or  cigars  a  day.  The  regime  should  under  no  circumstances 
be  so  strictly  adhered  to  as  to  cause  the  patients,  already  suffering 
from  irritating  conditions,  to  become  further  annoyed  thereby.  Diet 
has  relatively  little  power  to  modify  the  trouble  and  fussy  dietaries 
are  superstitious  nonsense  for  the  most  part. 

The  greatest  relief  from  rigidity  comes  from  the  regular  use  of 
passive  movements.  The  Zander  apparatus  can  be  utilized.  Working 
with  carpentry  or  garden  tools  is  often  very  helpful.  Heat  is  grateful 
and  helpful  and  passive  motion  combined  with  warm  (not  hot) 
baths  is  particularly  gratifying.    A  few  patients  react  badly  to  baths. 

Such  attempts  at  occupation  therapy  must  be  carefully  dosed. 
Fatigue  must  be  avoided.  Five  to  ten  minutes  is  sufficient  in  the  early 
stages.    Such  therapy  is  solely  of  value  from  a  psychical  stand-point. 

Drug  therapy  is  at  times  of  doubtful  service  in  controlling  the 
tremor.  The  remedies  are  those  with  marked  action  on  incoming 
nerve  impulses — notably  the  alkaloids  of  the  belladonna  group,  of 
which  hyoscin,  duboisin,  scopolamin,  and  atropin  are  the  most  avail- 
able. In  view  of  the  chemical  uncertainties  concerning  the  alkaloids 
of  this  group  one  should  obtain  good  products  and  try  the  different 
derivatives.    The  dosage  must  be  tested  with  each  case. 

The  analgesics,  particularly  in  combination  with  salicylates,  are  use- 
ful in  relieving  the  muscular  soreness  and  pain  of  tension — phenacetin, 
aspirin,  acetanilid  combinations,  etc. 

For  sleep,  the  best  form  of  hypnotic  is  not  yet  known.  Bromides 
are  at  times  available,  at  other  times  the  alcohol  hypnotics — trional, 
sulphonal,  again  urea  substitutes,  as  veronal — are  useful.  One  should 
avoid  morphin  as  much  as  possible,  particularly  bearing  in  mind  that 
the  emotional  hyperactivity  may  have  little  real  feeling  behind  it. 
It  is  often  mostly  mimicry  which  is  uncontrollable  because  of  the 
motor  defect. 

DYSTONIA   MUSCULORUM   DEFORMANS. 

Under  this  term,  Oppenheim  includes  a  peculiar  syndromy,  first 
called  attention  to  b}'^  Ziehen  as  a  tonic  torsion  neurosis.  Flatau  and 
Sterling  term  it  a  progressive  torsion  spasm.  It  is  preeminently  a 
disorder  of  children,  most  of  the  observed  patients  having  been  between 
eight  and  fourteen  years,  and  almost  all  of  the  Jewish  race.  Apparently 
there  are  no  sex  differences.  Oppenheim's  cases  did  not  lead  him  to 
any  light  on  the  disorder  as  regards  heredity.  Three  of  Ziehen's  cases 
were  brothers  and  sisters.  Exciting  or  other  causative  factors  are 
not  known. 

The  illness  comes  on  apparently  gradually  and  subtly  in  one  arm 


432     PARALYSIS  AGITANS,   CHOREA,   RELATED  DISORDERS 

or  in  both  arms,  occasionally  first  in  the  legs  or  in  the  spine,  but  in 
progression.  The  patient  twists  the  spine  in  a  peculiar  fashion,  tilting 
the  pelvis,  and  bringing  about  a  marked  torsion  of  the  entire  vertebral 
axis,  with  lordosis,  scoliosis,  and  tilting  of  the  pelvis,  the  arms  and 
legs  moving  in  a  peculiar  manner.  The  mode  of  progression  at 
times  resembles  that  of  astasia  abasia.  The  whole  musculature,  when 
in  action,  is  extremely  stiff  and  hypertonic;  when  at  rest,  hypotonia 
is  apparent.  In  the  general  attitude  of  the  patient  in  walking  one  is 
inclined  to  regard  the  whole  matter  as  one  of  extreme  suggestibility; 
a  psychogenic-hysteriform  affair.  The  movements  of  the  hip  are  very 
typical.     It  is  tilted  or  thrust  forward  or  backward  in  an  awkward 


Figs.  225  and  226. — Attitudes  in  dystonia  musculorum  cases.     (Flatau.) 


manner.  Oppenheim  has  likened  it  to  a  dromedary  in  some  of  the 
positions  assumed.  Walking  seems  to  fatigue  the  patients  greatly. 
They  perspire,  get  red,  and  show  signs  of  fatigue,  getting  out  of  breath, 
and  one  of  our  (J.)  patients  grunted  involuntarily.  One  of  Oppen- 
heim's  patients  could  walk  backward  better  than  he  could  walk  for- 
ward. On  sitting  down  or  lying  down  the  movements  cease  (Beling's 
case),  or  are  much  reduced  in  frequency  and  in  clownishness.  Ziehen's 
cases  were  at  times  continually  in  motion,  and  had  to  be  kept  in  a 
special  bed,  against  which  they  frequently  bruised  themselves  by  their 
impulsive  movements.  The  peculiar  activities  come  into  play  as  soon 
as  there  is  an  attempt  to  make  any  voluntary  movement.     Writing 


THE  CHOREAS  433 

becomes  difficult  or  impossible.  There  is  no  paralysis,  Oppenheim 
speaks  of  a  dystonia,  Ziehen  of  a  hypertonia.  The  movements  are  not 
athetoid  nor  choreic.  They  are  wide,  irregular,  and  yet  partake  of 
the  nature  of  both,  and  at  times  resemble  those  of  Huntington's  chorea. 

Tonic  and  clonic  extension  of  the  muscles,  particularly  of  the  biceps 
and  rotators  of  the  thigh,  were  marked  in  Oppenheim's  cases.  Thus 
there  is  an  alternation  in  tonicity  of  the  muscle. 

The  knee-jerks  are  apt  to  be  much  diminished,  coming  out  in 
Oppenheim's  cases  only  by  Jendrassik  reinforcement. 

The  relationships  are  difficult  to  state.  Hysteria  should  be  excluded. 
Double  infantile  athetosis  shows  a  similar  picture.  These  cases  have 
been  studied  especially  by  Lewandowsky.^  Cecile  Vogt  and  Oppen- 
heim have  reported  on  infantile  pseudobulbar  palsy,  which  also  is 
to  be  considered  in  this  connection.^  The  patients  apparently  hold 
their  own  for  some  time,  and  but  little  is  known  of  the  development 
of  the  disorder.  As  yet  no  pathological  reports  are  available.  No 
known  method  of  treatment  seems  useful.  Suggestion  is  of  no  value, 
nor  are  bromides.    The  movements  cease  during  sleep. 

THE  CHOREAS. 

The  choreas  are  due  to  definite  but  usually  recoverable  brain  dis- 
orders, chiefly  located  in  or  involving  the  cerebellar  static  mechanisms. 
They  are  a  vast  conglomeration  of  conditions,  certain  trends  of  which 
have  been  separated  out  under  a  variety  of  types. 

The  detailed  history  of  this  sorting  process  would  lead  too  far.  The 
chief  trends  center  about  the  study  of  the  movements  which  are 
present.  These  are  of  two  main  forms:  (a)  spontaneous  movements, 
and  (b)  coordination  disturbances.  In  most  choreas  the  two  forms 
are  present  but  in  varying  proportions. 

The  chief  diagnostic  entities  which  have  been  erected  are: 

1.  Chorea  Minor  or  Sydenham's  Chorea. — The  most  widespread  and 
frequent  of  the  trends,  usually  found  in  children  or  young  adults, 
and  transitory. 

2.  Chorea  Chronica. — A  stationary  form  of  the  former,  or  when 
occurring  in  old  age,  chorea  senilis. 

3.  Chorea  Huntington. — A  chronic  progressive  type  with  certain 
definite  hereditary  factors  and  one  showing  a  vast  variety  of  other 
choreic  anomalies  in  the  "  non-Huntington  chorea"  members. 

4.  Chorea  degenerans  of  Brissaud,  occurring  as  a  result  of  presenile 
breakdown  in  unstable  neuropathic  individuals. 

5.  Chorea  electrica  of  Dnbini,  an  acute,  usually  fatal  disturbance, 
often  occurring  with  epileptiform  attacks,  with  paralysis  and  death, 

6.  Chorea  electrica  of  Bergeron  and  Henoch,  occurring  in  young  per- 
sons, seven  to  fourteen  years,  with  rhythmic  lightning-like  movements 

1  Deutsche  Zeitsch.  f.  Nervenheilk.,  xxxix,  1908. 

2  Journal  f.  Neurologie,  xviii,  1911. 
28 


434     PARALYSIS  AGITANS,   CHOREA,   RELATED  DISORDERS 

of  the  neck,  shoulders,  and  upper  arms.  It  has  allies  in  certain  epileptic- 
like choreas. 

7.  Chorea  epileptica,  continuous  with  the  preceding  or  related  to 
cortical  epilepsies.    (See  Epilepsy.) 

8.  Choreas  of  general  paresis,  in  which  spontaneous  choreiform 
movements  occur.     (See  Paresis.) 

9.  Choreas  of  many  Psychoses. — Motility  psychoses  of  Wernicke, 
chiefly  schizophrenic  individuals,  in  whom  Kleist  has  endeavored  to 
show  an  involvement  of  the  cerebello-rubro-cortical  tracts. 

10.  Choreas  of  Congenital  or  Infantile  Cerebral  Palsies. — (See  Hemi- 
plegia, Thalamic  Syndrome.) 

11.  Chorea  Postapoplectica.  Posthemiplegic  Choreas. — (See  Hemi- 
plegia.) 

12.  Chorea  Thalamica. — (See  Thalamic  Syndrome.) 

13.  Choreas  due  to  Disorder  Involving  the  Superior  Cerebellar 
Peduncles. — (See  Cerebellar  Syndromes.) 

14.  Choreas  of  Cerebellar  Origins. 

15.  Chorea  tabica  in  tabes  with  choreic  crises  with  or  without  pain. 
Chorea  Minor  {Sydenham's  Chorea,  St.  Vitu^'  Dance)  is  the  most 

frequent  of  these  disturbances  of  spontaneous  and  coordinating  move- 
ments, which,  occurring  in  children,  usually  recover. 

Infection  and  maldevelopment  are  the  most  frequent  etiological 
factors.  The  most  widespread  infecting  agents  are  various  strep- 
tococci, hence  the  frequent  complications  of  infectious  arthritides, 
endocarditis,  with  a  mild  meningitis  which  is  usually  present. 

Girls  are  more  susceptible  to  these  infections,  and  hence  show  a 
higher  percentage  of  choreic  attacks. 

Symptoms. — These  show  considerable  variation,  ranging  from  slight 
motor  unrest  and  irritability  to  marked  motor  disturbances,  with 
corresponding  modifications  of  conduct  and  emotional  response. 
The  latter  at  times  are  so  severe  as  to  constitute  a  psychosis  (choreic 
mental  disease,  q.  v.).  The  motor  symptoms  are  best  grouped  as 
pyramidal  or  extrapyramidal  tract  and  cerebellar  disturbances,  either 
or  both  occurring  in  most  patients.  They  are  the  results  of  definite 
irritations,  occasionally  of  defect  (diaschisis)  lesions. 

The  spontaneous  movements  are  quick  and  show  comparatively  wide 
excursions.  In  the  distal  extremities  cramps  of  single  muscles  or  groups 
of  muscles  occur,  with  complete  rest  following  the  movements.  There 
is  great  irregularity  in  the  affected  groups;  there  will  be  one  or  two 
movements,  which  are  followed  by  opposite  muscle  action. 

In  mild  cases  these  movements  are  limited  to  the  face  and  to  single 
muscles.  In  the  more  severe  ones  the  entire  body  musculature  is 
involved. 

The  arms  are  flung  about,  the  legs  are  wobbled  and  pulled,  walking 
is  impossible,  the  larynx,  lips,  and  eyes  are  in  activity.  The  patients 
gasp,  snort,  and  groan.  In  the  mild  cases  the  movements  may  cease 
during  sleep;   in  the  more  severe  ones  the  movements  are  continuous. 


THE  CHOREAS  435 

Any  sensory  stimulus  may  increase  these  spontaneous  movements. 
They  do  not  behave  Hke  willed  movements.  Hence,  extrapyramidal 
systems  are  involved. 

Pyramidal  Tract  Disturhances. — Certain  choreic,  jerky  movements 
are  observed  apart  from  the  more  usual  ataxias  and  incoordinate  move- 
ments. The  latter  diminish  with  rest,  quiet,  and  relaxation;  the 
former  do  not  seem  to  diminish  as  muscle  activity  is  withdrawn. 

A  group  of  minor  signs  appear  on  close  examination.  One  of  these  is 
the  Babinski  hand  sign.  When  the  choreic  patient  extends  the  hand, 
palm  down,  one  side,  that  most  affected,  has  a  tendency  to  sag.  Or 
if  the  hands  are  hanging  by  the  side,  the  more  affected  hand  shows  a 
position  half-way  between  pronation  and  supination,  whereas,  since  the 
normal  muscle  tonus  is  greater  in  the  pronators,  the  more  healthy  side 
is  held  more  pronated.  This  is  in  accord  with  the  general  tendency 
for  one-half  of  the  body  to  be  more  affected  than  the  other.  The 
affected  side  is  hypotonic;  the  shoulder  droops  more.  There  is  apt 
to  be  exaggerated  flexion  or  extension  of  the  arms.  In  the  lower 
extremities,  hyperflexions  of  the  leg  on  the  thigh  occurs.  Since  hypo- 
tonicity  is  characteristic  of  underdeveloped,  psychomotor  integration, 
younger  children  do  not  bring  these  contrasts  into  relief. 

The  patient  lying  flat  upon  the  back  attempts  to  sit  up,  the  arms 
being  folded.  The  leg  most  affected  is  flexed  on  the  thigh  and  raises 
from  the  bed,  the  healthy  side  remains  flat.  (Babinski  thigh  sign — 
Hoover).  Similarly,  the  patient  lying  on  the  back  raises  the  legs, 
simultaneously,  the  sound  side  rises  higher  than  the  other,  or  if  one 
leg  is  raised  and  then  the  other,  the  sound  side  is  raised  higher  and  the 
choreic  side  also  falls  more  readily. 

The  Hoover  procedure  by  measuring  the  pressure  of  the  leg  on  the 
heel  during  the  movements  just  mentioned  shows  diminished  pressure 
on  the  weak  side.     (See  Examinations.) 

Thomas  showed  that  in  choreics,  particularly  in  the  more  unilateral 
cases,  the  closing  of  the  fist  of  the  sound  side  called  forth  an  associated 
closure  of  the  affected  side,  but  not  vice  versa.  Similar  associated 
abductor  or  adductor  movements  occur  in  the  lower  limbs. 

The  Babinski,  Oppenheim,  Gordon,  and  Chaddock  great-toe  exten- 
sion sign  is  very  frequent  in  chorea,  and  should  be  looked  for. 

Striimpell's  contraction  of  the  tibialis  anticus  occurs  when  the 
patient  lying  on  the  back  attempts  to  flex  the  leg  on  the  thigh  against 
passive  resistance  applied  to  the  thighs.  The  foot  assumes  the  equino- 
varus  position. 

The  tendon  reflexes,  patellar  and  Achilles,  are  apt  to  be  variable  and 
often  delayed. 

These  signs  are  all  suggestive  of  mild  types  of  hemiplegia,  and  have 
been  collected  under  the  title  of  the  "Little  Signs  of  Hemiplegia. "^ 
Someone  or  all  may  be  present  in  even  mild  cases  of  chorea,  especially 

1  Jelliffe,  Little  Signs  of  Hemiplegia,  Post  Graduate  Medical  Journal,  1912. 


436     PARALYSIS  AG  I  TANS,   CHOREA,   RELATED  DISORDERS 

when  the  disturbance  is  at  its  height  and  particularly  in  the  severe 
infectious  types.  Their  disappearance  often  takes  place  with  the 
stage  of  recovery.  Mild  cases  may  show  only  the  most  passing  signs, 
or  very  mildly  developed  ones. 

Cerebellar  Siffns. — These  are  chiefly  adiadokokinesia  and  asynergia. 
The  former  is  frequently  found,  especially  on  the  more  hypotonic 
side.    It  is  sometimes  complicated  by  the  choreic  movements.^ 

Asynergia  (dysmetria)  is  the  usual  choreic  type  of  movement.  It 
is  the  jerky,  irregular  movement  of  the  muscles  which  fail  to  perform 
well-adapted  movement.  Thus  the  patients  drop  things,  lurch, 
stumble  or  fall,  spill  their  food,  or  speak  in  a  jerky,  at  times  mumbling, 
manner.  They  are  incapacitated  from  writing,  playing  on  the  piano, 
or  for  making  any  finely  adaptive  movements.  The  finger-nose  and 
finger-finger  tests  show  this  dysmetria  by  the  overshooting  of  the 
mark,  pseudo-ataxia.  Attempting  to  grasp  an  object,  the  choreic 
opens  the  affected  hand  over  widely;  the  pencil  test  also  shows  similar 
overshooting.     (See  Figs.  211  and  212.) 

Leukocytes  are  usually  increased  in  the  cerebrospinal  fluid.  (See 
Meningeal  Inflammation.) 

Etiology  and  Pathogenesis. — A  too  narrow  view  of  the  causation  of 
chorea  has  prevailed.  While  many  patients  undoubtedly  develop  a 
chorea  following  streptococcus  or  other  infection,  this  whole  group 
should  be  looked  at  from  the  developmental  point  of  view  as  well.  This 
renders  fatigue  factors  comprehensible. 

The  so-called  hereditary  factor  is  probably  dependent  upon  an  infe- 
rior or  slowly  developing  psychomotor-cerebellar  integration.  In  cer- 
tain patients  the  rapidly  developing  body  calls  for  a  higher  grade  of 
motor  adaptation  than  the  developing  motor  integration  can  subserve. 
This  is  seen  normally  in  the  cerebellar  static  apparatus  in  what  is  so 
widely  called  the  awkward  age,  or  in  the  "puppy"  stage,  where  the 
analogues  to  mild  choreic  affection  are  obvious.  Chorea  may  thus 
develop  in  adolescents  from  the  mildest  of  infections  or  even  from 
excess  of  motor  activity.    Thus  chorea  may  be  a  fatigue  symptom. 

Ancestral  syphilis  is  responsible  for  certain  choreas,  especially  as 
affecting  the  full  development  of  the  nervous  system,  thus  allying 
such  choreas  with  related  spinal,  cerebellar,  and  cortical  ageneses 
(Friedreich,  etc.).  Hence,  the  Wassermann  tests  should  be  applied 
in  chorea  diagnosis  (provocative  test  desirable).  Milian  has  found 
from  60  to  70  per  cent,  of  flfteen  choreics  examined  to  show  positive 
Wassermann  reaction. ^  This  may  account  for  the  good  results  from 
salvarsan  in  the  treatment  of  some  choreas. 

Acute  rheumatic  infection  loses  much  of  its  so-called  specificity. 
Tonsillitis  is  also  assuredly  not  the  universal  malefactor.  These  and 
other  infections  are  important  as  reducing  physiological  efficiency,  by 
interfering  with  the  fiber-carrying  capacity   (through  inflammatory 

1  Grenet  et  Loubet,  Rev.  Neur.,  December  15,  1912,  p.  632. 

2  Milian,  Soc.  Med.  d.  Hop.,  November  29,  1912. 


THE  CHOREAS  437 

exudate  at  times),  especially  in  some  cases  where  meningitis  is  obvious, 
of  an  insufficient  or  tardily  maturing  and  integrating  motor  apparatus, 
especially  in  its  cerebellar  static  portion. 

Diagnosis. — The  chief  disorders  to  be  excluded  are  hysteria,  fre- 
quently from  imitation,  and  the  more  persistent  and  milder  of  the 
compulsive  tics.  The  organic  signs  enumerated  will  be  found  in  the 
majority  of  choreics,  if  carefully  looked  for.  Lumbar  puncture  may  be 
resorted  to  in  difficult  cases,  although  those  with  definite  leukocytosis 
are  apt  to  show  the  organic  signs. 

The  diagnosis  of  hysteria  will  depend  upon  the  finding  of  definite 
conversion  mechanisms,  while  that  of  the  tics  will  develop  substitu- 
tions. The  movements  in  both  may  be  similar  to  these  called  choreic, 
but  in  both  hysteria  and  in  tics  hypotonus  is  less  liable  to  show.  There 
is  a  greater  likelihood  that  so-called  hysterical  movements  will  turn 
out  to  be  choreas  than  the  reverse,  especially  in  younger  children. 
In  older  children  or  in  young  adults  the  opposite  may  be  more  seriously 
entertained. 

Treatment. — The  best  treatment  is  rest  in  bed,  with  partial  isolation, 
no  playing,  local  treatment  of  infectious  areas,  full  diet,  with  increased 
fatty  ingredients  (milk  and  eggs),  and  intravenous  use  of  neosalvarsan 
in  some  cases. 

The  rest  in  bed  should  be  practically  absolute  for  a  week  or  two 
before  the  use  of  arsenic. 

One  may  start  with  milder  arsenical  preparations  in  less  severe 
attacks,  and  in  those  where  sudden  disproportionate  growth  seems  to 
play  a  larger  role,  Fowler's  solution  TTLv-xv,  or  the  acidi  arsenosi,  may 
be  used,  either  alone  or  in  pleasing  vehicles. 

In  severe  and  protracted  cases  the  intravenous  use  of  doses  of  0.05 
to  0.2  gm.  of  salvarsan,  according  to  age,  once  a  week  for  four  weeks, 
is  advantageous. 

Most  of  the  mild  cases  recover  on  prolonged  rest  in  bed,  without 
medication,  if  on  a  full  diet,  with  milk  and  eggs  in  abundance. 

Huntington's  Chorea. — This  is  a  disorder  of  the  nervous  system, 
named  after  George  Huntington  (born  1850),  an  American  physician, 
who  gave  the  first  essentially  comprehensive  and  distinctive  descrip- 
tion of  the  disease.  Huntington's  grandfather  and  father  had  observed 
the  disorder  in  one  of  its  American  foci,  Easthampton,  Long  Island, 
and  chiefly  through  their  studies  of  several  generations  of  afflicted 
families  the  essentially  hereditary  nature  of  the  malady  became 
apparent. 

Huntington's  chorea  has  no  relationships  to  Sydenham's  chorea, 
that  essentially  infantile  disturbance  of  cerebrocerebellar  tract  coor- 
dinations following  so  frequently  upon  infectious  disease  or  exhaustion. 
Huntington's  chorea  is  essentially  hereditary  and  chronic,  occurring, 
as  a  rule,  in  adults  from  thirty  to  fifty  years  of  age. 

The  condition  did  not  escape  earlier  observers.  Thilenius  gave  a 
report  of  a  case  apparently  as  early  as  1816.    Rufs,  another  in  1834. 


438     PARALYSIS  AGITANS,  CHOREA,  RELATED  DISORDERS 

Waters  made  his  striking  comment  in  1841.^  Dr.  Charles  G.  Gorman, 
of  Luzerne,  Pa.,  wrote  an  inaugural  thesis,  in  1848,  on  this  affection, 
which  has  been  lost.  Dr.  Irving  W,  Lyon,  while  house  physician  at 
Bellevue  Hospital,  wrote  a  paper  on  "Chronic  Hereditary  Chorea," 
which  was  published  in  the  American  Medical  Times  in  1863.  Hunting- 
ton's paper  appeared  in  the  Medical  and  Surgical  Reporter,  Phila- 
delphia, in  1872.  Since  this  time  a  rich  bibliography  has  accumulated, 
which  in  the  Huntington  number  of  Neurographs,  edited  by  Dr. 
William  Browning,  in  1908,  mounted  to  200  titles. 

Etiology. — So  far  as  is  known  the  disorder  is  hereditary.  From 
eugenic  studies  of  Davenport,  Muncie,  and  Jelliffe,^  the  chief  factors 
or  determinants  behave  as  Mendelian  dominants.  Heilbronner  has 
said  that  the  disorder  appears  at  later  intervals  in  succeeding  genera- 
tions, but  evidence  of  the  extensive  eugenic  studies,  which  include  the 
study  of  1000  cases  of  Huntington's  chorea,  limited  to  a  few  families, 
shows  that  it  appears  at  earlier  years  in  succeeding  generations.  The 
data  here  indicated  that  the  disease  behaves  as  a  complex  in  which 
age,  motor  disturbances,  and  mental  defect  behave  more  or  less 
independently  one  of  another.  When  all  three  factors  combine,  the 
result  is  Huntington's  chorea.    No  other  etiology  is  known. 

Symptoms. — As  it  is  not  possible  in  a  short  resume  to  discuss  the 
separate  factors,  the  older  lines  of  description  which  regard  the  dis- 
order as  a  unit  will  be  followed.  Thus  one  speaks  of  an  insidious  onset, 
usually  coming  on  between  the  years  of  thirty  and  forty.  The  earlier 
signs  are  either  slight  changes  in  character,  irritability,  moroseness, 
eccentricities,  or  the  choreic  movements  become  prominent  in  the 
picture.  The  facial,  neck,  and  upper  extremity  muscles  are  usually 
involved  first.  There  are  involuntary,  jerky  movements,  usually  of 
muscle  groups,  not  of  muscle  fibers.  The  excursions  brought  about  are 
massive,  i.  e.,  excessive,  loose,  and  hypotonic.  The  hand  is  thrown  to 
one  side,  the  whole  arm  sweeping  outward;  the  neck  is  jerked  back- 
ward, the  head  bowed  forward  in  a  quick,  loose-jointed,  jerky  sort  of 
way.    The  motor  unrest  spreads  over  the  entire  body. 

The  patient,  after  several  years — for  the  motor  disturbance  advances 
slowly — becomes  jerky,  and  although  for  a  long  time  voluntary 
movement  is  able  to  check  the  excessive  motion,  finally  control  is  lost 
and  the  patient  is  confined  to  a  chair  or  his  bed,  making  his  peculiar 
broad,  jerky  movements.    During  sleep  the  motions  cease. 

Nearly  all  of  the  voluntary  muscles  may  be  involved.  The  eye 
movements  seem  to  resist  to  the  last.  The  speech  becomes  explosive, 
or  grunt-like,  very  incoherent  at  times  by  reason  of  the  involuntary 
movements  of  tongue,  lips,  diaphragm,  and  chest.  Even  swallowing 
is  involved  in  the  later  stages.  Writing  soon  becomes  impossible  by 
reason  of  the  jerkiness  of  the  hands  and  arms.     Walking  becomes 

1  Dunglison's  Practice  of  Medicine,  ii,  312. 

2  American  Neurological  Association,  1913. 


THE  CHOREAS  439 

successively  more  and  more  unsteady  until  the  patient  becomes  bed- 
ridden. 

There  are  few  disturbances  of  sensibility  and  these  are  observed  only 
late  in  the  deteriorated  phases.  The  knee-jerks  are  active  or  unin- 
volved;    there  are  no  atrophies,  nor  paralyses,  nor  hypertrophies. 

The  mental  changes  may  develop  apart  from  the  motor  ones,  and 
in  choreic  families  mental  choreics  are  to  be  recognized  who  perhaps 
may  never  show  motor  signs  or  those  who  show  choreic  movements 
very  late.  This  is  the  basis  of  Diefendorf's  constitutionally  defec- 
tive group.  These  patients  even  in  childhood  may  be  excessively 
nervous,  irritable,  and  excitable.  They  are  often  difficult  children  to 
manage.  Their  eccentricities  become  more  and  more  marked  with 
advancing  years.  Some  show  marked  grades  of  feeble-mindedness  and 
occasionally  are  born  choreic  and  defective. 

In  the  great  majority  of  the  cases  the  insidious  and  slow  develop- 
ment of  great  instability  and  irascibility  shows  itself  coincident  with 
or  following  the  choreiform  movements.  Angry  outbursts  and  destruc- 
tive impulses  occur,  often  followed  or  preceded  by  periods  of  marked 
moroseness  and  despondency.  This  depression  or  gloom  may  be  a 
forerunner  of  suicide.  Diefendorf  remarks  that  this  despondency  is 
not  due  entirely  to  the  realization  of  having  the  disease.  With  some 
patients,  however,  the  suicide  is  to  be  traced  to  the  knowledge  of  the 
taint.  Suspiciousness,  paranoid  ideas  and  jealousies  are  not  infre- 
quent mental  signs.  Emotional  deterioration  follows.  The  patient 
loses  all  interest  in  his  work,  his  appearance,  his  home,  etc.  Some 
become  tramps.  Intemperance  and  free  sexual  activities  may  show 
themselves  with  this  gradual  deterioration.  Indifference  shades  off 
into  absolute  incapacity  and  deterioration  becomes  profound,  always, 
however,  showing  itself  in  the  affective  sphere  more  prominently  than 
in  the  intellectual  capacities,  although  these  latter  are  not  free  from 
gross  defect;  the  patients  being  forgetful,  poor  in  ideas,  disorderly 
in  thought  and  weak  in  judgment. 

Diefendorf  speaks  of  a  group  in  which  the  mental  symptoms  develop 
somewhat  similarly  to  those  seen  in  the  hebephrenic  types  of  dementia 
precox.  These  patients  complain  of  insomnia  and  general  malaise. 
They  often  then  develop  ideas  of  reference,  anxiety,  suspiciousness 
and  ideas  of  infidelity.  In  some  of  these  impulsive  activities  show 
themselves.  Homicide  has  taken  place.  Kraepelin  cites  an  illustra- 
tion of  a  choreic  father  who  killed  his  three  small  children  by  hanging, 
as  he  feared  he  could  not  support  them,  then  quietly  took  a  walk  and 
was  quite  unconcerned  about  the  affair  at  a  judicial  hearing.  The 
eating  is  often  impulsive  and  ravenous.  In  some  of  these  cases,  as 
with  the  inferior  group,  the  mental  symptoms  may  develop  long  before 
the  choreic  symptoms,  and  the  diagnosis  of  a  katatonic  schizophrenic 
may  be  made  as  the  motor  symptoms  become  manifest.  Here  the 
diagnostic  difficulties  are  very  definite. 

It  would  appear  from  Diefendorf  and  the  studies  cited  that  the 


440     PARALYSIS  AGITANS,   CHOREA,   RELATED  DISORDERS 

mental  and  motor  traits  are  more  or  less  independent  one  of  the  other. 
In  inheritance  they  seem  to  show  as  such.  Some  patients  have  shown 
choreic  movements  for  twenty  to  thirty  years  without  mental  signs. 

Course. — This  is  subject  to  great  variation.  Often  the  patients  sud- 
denly develop  great  motor  unrest;  the  mental  signs  augment  rapidly, 
and  the  patient  dies  in  exhaustion  five,  ten  or  fifteen  years  after  the 
onset  of  the  symptoms.  Usually  the  disorders,  i.  e.,  motor  and  mental, 
are  progressive;  rarely  they  may  diminish  after  reaching  a  severe 
grade.     Many  die  of  intercurrent  disease. 

Pathology. — No  unitary  interpretation  seems  yet  possible.  The  motor 
and  the  mental  symptoms  are  best  considered  separately. 

A  number  of  autopsies  have  shown  a  variety  of  findings.  At  times 
there  are  chronic  meningeal  thickenings,  again  generalized  brain 
atrophy.  This  reduction  of  the  cortex  may  show  to  a  marked  degree 
in  the  loss  of  cells,  particularly  of  the  third  layer.  There  is  a  compen- 
satory increase  in  neuroglia.  In  some  patients  arteriosclerosis  has  been 
present,  in  others  not.  The  older  patients  showed  the  arteriosclerotic 
changes.  These  cortical  cellular  changes  are  apparently  more  frequent 
in  the  frontal  areas.    They  are  Correlated  with  the  mental  defects. 

The  pathology  behind  the  motor  manifestations  is  more  obscure. 
Theoretical  considerations  as  well  as  pathological  findings  point  to  an 
implication  of  the  rubro-thalamo-cortical  extensions  of  the  cerebellar 
pathways  as  chiefly  responsible  for  the  perverted  movements.  Kleist, 
Zingerle,  Jelgersma,  and  Winkler  adduce  observations  from  various 
sides  which  tend  to  show  that  these  mechanisms  are  involved.  Numer- 
ous autopsies  show  changes  in  the  lenticular  region  which  may  be 
taken  to  support  these  contentions.  Thus  the  motor  signs  have  a 
pathology  similar  to  that  seen  in  paralysis  agitans  and  other  midbrain 
tremors. 

Therapy. — There  is  no  efficient  therapy.  Many  patients  need  hos- 
pital care.  Prophylaxis  is  important,  Mendelian  dominance  arguing 
for  certain  factors  at  least,  that  these  patients  should  not  procreate. 
The  percentage  of  chance  of  escape  for  Huntington  choreics  is  about 
one  in  four  at  the  best.  Some  branches  escape,  and  a  branch  once 
free,  is  always  free.  Only  a  few  exceptions  to  this  are  found  in  the 
Davenport,  Muncie,  and  Jelliffe  extensive  series. 


CHAPTER  XI. 

DISEASES  OF  THE  MENINGES. 

Here  diseases  of  the  dura,  the  arachnoid,  and  the  pia  are  to  be 
recognized.  Under  the  first  various  types  of  meningeal  hemorrhage 
and  inflammation — pachymeningitis — are  found.  Under  the  latter 
various  forms  of  leptomeningitis  and  cerebrospinal  meningitis. 

DURAL   DISEASE. 

1.  Meningeal  Apoplexy  (Traumatic  Meningeal  Hemorrhage). — 
Etiology. — Trauma  from  blows,  instruments,  violence  of  various 
kinds,  causes  either  a  rupture  of  the  branches  of  the  median  meningeal 
artery,  the  veins,  or  of  the  cerebral  sinuses.  Such  hemorrhages  may 
be  found  at  the  site  of  the  injury,  or  at  the  point  opposite. 

The  hemorrhage  may  be  extradural,  between  the  pia  and  dura, 
epidural,  or  between  the  pia  and  cortex,  arachnoidal  hemorrhage. 
The  bleeding  may  be  diffuse  or  circumscribed.  At  childbirth  such 
hemorrhages  with  partial  destruction  of  the  brain  substance  itself 
are  extremely  frequent.  Here  the  bleeding  is  almost  always  exclusively 
venous. 

Pathology.  —  Macroscopically  such  hemorrhages  resemble  hemor- 
rhagic pachymeningitis,  but  microscopically  they  differ,  especially  in 
the  absence  of  new  elements — vessels,  plasma  cells,  etc. 

Not  infrequently,  in  severe  fractures,  the  brain  substance  is  also 
involved. 

Symptoms. — These  will  vary  according  to  the  severity  of  the  causing 
lesion,  the  amount  of  blood  thrown  out,  and  the  site  and  extent  of 
the  bleeding.  In  severe  injuries  there  are  signs  of  shock  and  concussion 
in  addition  to  the  symptoms  of  pressure.  Unconsciousness  becomes 
increasingly  deeper,  the  pulse  is  slow  in  the  beginning,  then  hastens, 
vomiting  takes  place,  urination  and  defecation  are  involuntary, 
irregular  respiration,  with  increasing  temperature,  and  death  often 
results  unless  operation  relieves  the  pressure. 

In  less  severe  lesions  the  initial  symptoms  of  concussion  with  vary- 
ing grades  of  stupor  partially  clear  up  for  an  hour  or  more,  even 
twenty-four,  or  a  few  days.^  Then  compression  symptoms  develop, 
with  signs  of  excitement.  Irritation  and  paralytic  signs  appear. 
There  may  be  spasms,  epileptiform  convulsions,  often  of  Jacksonian 

1  Connell,  Free  Interval  in  Meningeal  Hemorrhages,  Surg.,  Gyn.  and  Obstet.,  March, 
1906. 


442 


DISEASES  OF  THE  MENINGES 


type,  monoplegias,  hemiplegias,  the  arm  often  suffering  the  worst. 
The  hemiplegia  is  usually  on  the  side  opposite  to  the  site  of  injury, 
but  in  about  5  per  cent,  of  the  cases  is  found  on  the  side  of  the  lesion 
(uncrossed  pyramids  or  contra  coup).  Aphasias  are  not  infrequent  in 
left-side  injuries.  The  tendon  reflexes  are  usually  increased  on  the 
paretic  side,  while  the  skin  reflexes  are  usually  diminished.  The 
Babinski  phenomenon  is  frequent  on  the  paralyzed  side,  and  occasion- 
ally present  on  the  side  of  the  hematoma.  Occasionally  hemianesthesia 
and  hemianopsia  can  be  made  out. 

Bleeding  at  the  base  may  show  involvement  of  the  cranial  nerves; 
occasionally  choked  disk  is  present. 


Fig.  227. — Pia-arachnoid  hemorrhage  from  contra  coup.     (Larkin.) 


The  pupils  vary  greatly.  Wiesmann  has  shown  in  70  cases,  that  in 
39  both  pupils  were  dilated  and  immobile,  in  20  there  was  dilatation 
on  the  side  of  the  hemorrhage,  in  7  both  pupils  were  small,  and  in  4 
there  was  dilatation  on  the  side  opposite  the  lesion. 

In  birth  hemorrhages,  Seitz  has  shown  that  sub-tentorial  hemor- 
rhages behave  differently  from  convexity  hemorrhages.  In  the  former 
the  children  frequently  show  no  signs  of  asphyxia,  then  after  a  few 
hours  respiratory  disturbances  set  in,  the  breathing  becomes  irregularly 
quickened,  with  spasms  and  cyanosis.  Then  spasms  of  the  eyeballs 
occur,  less  often  facial  spasms.  If  the  pressure  is  directed  downward 
toward  the  medulla,  opisthotonus  and  muscular  rigidity  develop,  and 
not  infrequently  priapism. 


MENINGEAL  HEMORRHAGE 


443 


In  convexity  hemorrhages  the  child  shows  considerable  restlessness, 
refuses  to  suckle,  and  then  develops  signs  of  brain-pressure,  pressure 
in  the  fontanelles,  respiratory  disturbances,  drowsiness  to  unconscious- 
ness, with  some  slowing  of  the  pulse.  Localizing  symptoms  may  then 
show  themselves,  spasms  of  the  opposite  arm  and  leg,  increased  tendon 
reflexes,  and  slight  hypertonus. 

Diagnosis. — It  is  extremely  difficult  to  determine  whether  one  has 
to  deal  solely  with  a  pure  meningeal  apoplexy,  or  whether  there  is 
also  intracerebral  disturbance.  If  there  is  a  definite  free  interval  after 
the  initial  signs  of  concussion,  with  the  gradual  or  sudden  onset  of 
compression  signs,  the  probabilities  are  for  hematoma  (80  per  cent.). 


Fig.  228. — Traumatic  supradural  hemorrhage.     (Larkin.) 

The  length  of  free  interval  offers  no  certain  criterion  as  to  site  of 
hemorrhage.  Choked  disk,  often  transitory,  also  speaks  for  hematoma. 
Bloody  spinal  fluid,  which  does  not  clot,  speaks  for  intradural  hemor- 
rhage, occasionally  extradural,  Neisser's  brain  puncture  and  lumbar 
procedures  often  help  in  clearing  up  a  diagnosis.  Long  intervals 
speak  for  abscess. 

Therapy. — This  is  surgical  and  should  be  immediate.  The  exact 
procedure  must  be  determined  largely  by  the  symptoms.  Even  the 
intracranial  hemorrhages  of  childbirth  may  be  controlled  by  skilled 
surgical  measures.  The  results  of  surgical  interference  are  three  times 
as  good  as  leaving  the  patients  alone. 


444  DISEASES  OF   THE  MENINGES 

Traumatic  late  apoplexy  is  a  special  variety  in  which  degenerative 
processes  complicate  the  picture.  The  patients  may  develop  the 
signs  of  hemorrhage,  usually  intracranially,  even  months  after  the 
injury.  Such  cases  are  to  be  diagnosed  with  much  caution.  Senility, 
pronounced  arteriosclerosis,  and  syphilis  should  be  excluded.^ 

2.  Inflammation  of  Dura  {Pachymeningitis) . — (^4)  Pachymeningitis 
Externa. — Following  severe  trauma,  purulent  processes  of  the  frontal 
sinuses,  the  middle  ear,  mastoid,  erysipelas,  caries,  or  osteomyelitis, 
one  occasionally  observes  an  inflammation  of  the  external  surface  of 
the  dura  of  the  cerebrum.  It  is  usually  localized.  A  similar  process 
may  be  present  in  the  spinal  dura. 

Symptoms. — These  are  usually  hidden  in  those  of  the  causative 
lesion  and  vary  with  the  acuteness  and  extent  of  the  process.  Localized 
cerebral  or  spinal  pains,  muscular  twitching,  spasms,  marked  scalp 
tenderness  to  percussion,  and  slight  rise  in  temperature  are  the  chief 
signs.  At  times  symptoms  of  localized  pressure  of  the  cerebrum  or  of 
the  cord,  resembling  tumor,  are  observed. 

Treatment. — The  treatment  is  that  of  the  causative  factors. 

{B)  Pachymeningitis  Interna  Simplex. — This  may  consist  of  a 
localized  or  circumscribed  purulent  exudate,  giving  in  the  main  the 
symptoms  of  a  brain  abscess  or  a  brain  tumor.  In  rare  instances 
(pneumonia,  etc.),  a  secondary  pseudomembranous  productive 
inflammation  occurs. 

(CO  Pachymeningitis  Interna  Hemorrhagica. — This  consists  of  a 
chronic  inflammation  in  the  internal  layers  of  the  dura,  associated 
with  extravasation  of  blood.  It  is  by  no  means  infrequent^  and  gives 
rise  to  chronic  psychoses  of  an  ill-defined  type. 

History. — Morgagni  noted  the  affection  in  the  eighteenth  century. 
Baillarger^  followed  the  older  authors  in  assuming  it  to  be  a  primary 
hemorrhage,  with  new  membrane  tissue  formation.  Heschl  and  Vir- 
chow^  first  pointed  out  the  infiammatory  nature  of  the  productive 
inflammation,  and  the  consequent  hemorrhage  due  to  the  rich  forma- 
tion of  new  bloodvessels.  Jores  and  modern  authors  support  these 
views. 

Occurrence,  Pathology. — Usually  a  disorder  of  advanced  years, 
hemorrhagic  pachymeningitis  may  be  found  in  children.^ 

It  is  extremely  frequent  in  general  paresis,  and  Blackburn  has 
found  it  to  cause  chronic  excited  and  chronic  depressed  states,  in 
patients  running  a  course  resembling  senile  dementia.  It  is  fre- 
quently an  alcoholic  complication.  It  is  seen  in  some  chronic  choreic 
affections.  Tuberculosis,  nephritis,  leukemia,  scorbutus.  Barlow's 
disease,  and  hemophilia  have  seemed  to  stand  in  causal  relations  in 
some  instances. 

1  Stadelmann,  Deut.  med.  Woch.,  1903;  Allen,  Jour.  Nerv.  and  Ment.  Dis.,  1909. 

2  Blackburn,  Government  Hospital  Autopsy  Reports. 

^  Maladies  du  Cerveau,  1854.  ■•  Wurzburger  Verhandlungen,  1856,  p.  134. 

5  Goppert,  Jahr.  f.  Kinderheilk.,  1905,  Ixi,  51;  Freund,  Monat.  f.  Kinderheilk., 
1909,  vii. 


HEMORRHAGIC   PACHYMENINGITIS  445 

In  the  initial  stages  a  productive  inflammation  causes  the  formation 
of  a  thin,  dehcate,  yellowish-brown  membrane  on  the  inner  surface 
of  the  dura.  The  base  is  rarely  affected,  the  temporal  regions  more 
often.  New  bloodvessels  form,  whose  w^alls  give  way,  giving  rise  to 
extravasation  of  blood.  The  process  of  new  membrane  formation  and 
bleeding  goes  on  hand  in  hand  until  the  whole  membrane  may  be  one 
or  more  centimeters  thick,  compressing  the  brain,  with  which  it  is 
usually  closely  united  by  new  connective-tissue  formation  and  new 
bloodvessels,  and  causing  atrophies,  degenerations,  softening,  or  scle- 
roses of  the  near-lying  portions  of  the  cortex.^  The  process  may  extend 
to  the  spinal  cord. 

Symptoms. — The  disorder  may  be  present  for  years  without  symp- 
toms. In  paresis  it  may  not  add  any  definite  symptoms  to  the  under- 
lying disorder,  or  it  may  cause  a  number  of  complicating  pressure 
pictures.  When  the  process  has  advanced  to  a  definite  extent  both 
general  and  local  symptoms  show  but  are  so  indefinite  as  to  defy  diag- 
nosis. Gradually  increasing  head  discomfort,  headache,  often  severe, 
some  nausea  or  vomiting,  irregular  periods  of  confusion  or  distress 
in  the  head,  with  at  times  mild  delirious  excitement,  are  among  the 
more  characteristic  earlier  signs. 

With  rapid  extravasations,  acute  pressure  symptoms  may  develop, 
with  epileptiform  convulsions,  hemiparesis,  comatose  states  with  slow 
pulse,  aphasias,  astasia-abasia.  It  is  very  characteristic  that  these 
may  be  transitory.  Persistent  monoplegias,  with  Jacksonian  attacks 
involving  irregularly'  distributed  muscle  groups  may  be  early  signs. 
Often  persisting  stereotyped  muscle  movements,  chewing,  automatic 
arm,  hand,  or  leg  movements  may  be  the  signs  of  a  local  irritative 
lesion.  Constantly  putting  the  hands  to  the  head  was  considered  sug- 
gestive of  pachymeningitis  by  Fiirstner.  It  is  a  frequent  sign  in 
paresis.  The  eye  muscles  are  rarely  involved  (conjugate  deviations, 
nystagmus),  and  still  more  rarely  one  finds  involvement  of  the  cranial 
nerves  at  the  base. 

Papillitis,  or  choked  disk,  is  often  present.  The  pupils  are  not 
infrequently  irregular,  occasionally  myotic  in  the  early  stage,  dilated 
in  coma,  and  at  times  immobile  to  light  and  accommodation.  Argyll- 
Robertson  may  be  present  with  or  without  positive  W^assermann. 
Increased  tendon  reflexes  show  the  presence  of  irritation  of  the  motor 
cortex,  clonus  and  Babinski  being  occasionally  present,  and  at  times 
coming  and  going.  Irregular  but  inconstant  temperature  changes 
are  often  present. 

Course. — This  varies  considerably,  is  usually  chronic,  shows  remis- 
sion and  exacerbations.  At  times  the  patient  recovers  completely, 
again  the  disorder  is  progressive,  causes  death  after  a  long  psychotic 
period  of  irregular  excitement  or  depression. 

1  Jores  u.  Laurent,  Ziegler's  Beitrage,  xxix. 


446  .         DISEASES  OF  THE  MENINGES 

Diagnosis. — It  must  be  separated  as  a  primary  and  as  a  secondary 
process  in  alcoholism,  paresis,  cerebrospinal  syphilis,  etc.  Brain  tumor, 
abscess,  hydrocephalus,  sinus  thrombosis,  leptomeningitis,  apoplexy 
are  to  be  borne  in  mind.  Traumatic  meningitis  must  be  excluded 
if  an  antecedent  trauma,  even  of  mild  grade,  such  as  falling  from  the 
bed,  in  bath  tub,  hard  crack  on  the  head  from  running  into  door,  etc., 
should  have  occurred. 

Neisser's  procedure  of  brain  puncture  is  always  desirable  in  those 
patients  that  give  signs  of  brain  tumor,  brain  abscess,  etc. 

Therapy. — In  acute  progressions,  local  bleeding  is  advisable.  Hydro- 
therapy with  stimulation  of  elimination  is  useful.  Brain  puncture  has 
been  of  service,  also  lumbar  puncture  in  children.  Abstinence  from 
alcohol  is  imperative.  Mercury  in  the  positive  Wassermann  cases 
is  indicated.  The  pains  are  often  relieved  by  analgesics,  and  by  warm 
baths.    Brain  puncture  with  injections  of  gelatin  have  been  successful. 

DISEASES    OF  THE  ARACHNOID  AND  PIA.     LEPTOMENINGITIS. 

1.  Acute  Leptomeningitides  (Cerebrospinal  Meningitides).  —  The 
studies  of  recent  years  have  shown  a  host  of  causes  for  acute  inflam- 
mation of  the  cerebral  and  spinal  arachnoid  and  pia.  These  vary 
very  widely  as  to  severity — simple  meningism  to  the  gravest  forms  of 
general  meningeal  involvement — epidemic  cerebrospinal  meningitis, 
epidemic  polio-myelo-encephalitis,  general  syphilitic  meningomyelitis, 
etc.  It  becomes  impossible  to  present  a  logical  classification  of  these 
disorders,  either  from  the  etiological,  pathological,  or  clinical  stand- 
point. In  general  only  the  more  circumscribed  types  of  meningitis 
will  be  considered  here.^ 

Causes. — These  are  many.  Trauma  is  one  of  the  most  important. 
Such  trauma  may  act  either  through  direct  infection,  as  by  compound 
fracture,  or  secondarily  bring  about  a  septic  meningitis,  through 
hemorrhage,  thrombosis,  and  subsequent  infection. 

Traumatic  early  and  late  meningitis  appear.  Many  tuberculous 
leptomeningitides  arise  from  trauma.  Occasionally  one  meets  with 
late  purulent  meningitides,  due  to  old  encapsulated  abscesses,  old 
projectiles  (bullets,  splinters,  etc.),  old  fractures  of  lamina  cribrosa. 

Infection  from  suppurative  processes  in  the  neighborhood  is  one 
of  the  most  frequent  causes  of  the  type  of  meningitides  under  con- 
sideration, and  chief  of  these  is  otitic  meningitis.  Suppurative  otitis 
may  lead  to  intra-  or  extracranial  abscess,  sinus  phlebitis,  thrombosis, 
direct  contagion,  contagion  through  labyrinth,  through  mastoid,  etc. 
Serous  meningitis  may  also  have  an  otitic  origin. 

Nasal  and  frontal  sinus  infection  gives  rise  to  a  small  number  of 
these  meningitides;  they  may  be  purulent  or  serous.  Operations  upon 
the  nose  are  frequently  complicated  by  meningitis. 

1  Recent  monographs  are  by  Schultze  (Nothnagel)  and  Finkelnburg,  Handbuch  d. 
Neurologic,  ii,  1090,  in  both  of  which  complete  bibliographies  are  to  be  found. 


LEPTOMENINGITIS  447 

Facial  erysipelas  occasionally  is  a  cause;  rarely  facial  furunculosis. 

Specific  organisms  give  rise  to  specific  types  of  meningitis.  Among 
these  are  measles,  scarlet  fever,  varicella,  typhoid,  diphtheria.  The 
influenza  bacillus  is  an  extremely  important  organism  in  this  respect, 
often  giving  rise  to  severe  epidemics  of  meningitis.  Malaria,  yellow 
fever,  anthrax,  leprosy,  actinomycosis,  pneumonia,  whooping-cough, 
each  may  be  the  exciting  factor.  Septic  extension  from  acute  articular 
rheumatism  is  a  factor.  Tuberculosis  is  a  large  item.  The  epidemic 
type  due  to  the  Diplococcus  intracellularis  is  one  of  the  most  character- 
istic of  the  types.  Old  abscess  formations  in  the  lung,  liver,  bladder, 
gonorrhea,  etc.,  all  may  give  rise  to  a  meningitis. 

Occasionally  one  ascribes  certain  meningitides  to  chemical  poisons: 
diabetes,  lead,  gout,  are  among  these. 

Symptoms. — These  show  certain  variations  according  to  the  mode 
of  infection  and  the  type  of  infecting  agent.  Since  simple  purulent 
meningitis,  tuberculous  meningitis  (usually  a  mixed  infection)  and 
epidemic  cerebrospinal  meningitis  are  the  chief  infections,  the  following 
description  will  apply  to  these  and  no  attempt  will  be  made  to  cover 
the  symptomatology  of  the  entire  group. 

Headache.— TYns,  is  one  of  the  earliest  signs,  and  is  frequent,  usually 
increasing  in  intensity  as  the  disease  progresses.  It  is  mostly  diffuse, 
but  may  first  appear  in  the  occipital  region,  or  in  the  forehead.  The 
slightest  movement,  percussion,  etc.,  increases  it. 

Stiffness  of  Neck.- — This  is  a  striking  symptom  and  develops  early, 
sometimes  preceding  the  headache.  Such  early  stiffness  may  or  may 
not  be  apparent  to  the  patient,  but  comes  out  on  careful  examination, 
and  is  not  infrequently  accompanied  by  painful  pressure  points  over 
the  cervical  vertebrae.  When  well  developed  the  least  movement  of 
the  neck  is  painful  and  the  attitude  of  the  patient  on  movement  is 
striking.  In  young  children  it  is  not  infrequently  absent,  and  in  adults 
in  chronic  cases.  It  is  a  further  characteristic  that  this  stiffness  and 
painfulness  shows  considerable  variation  even  during  the  twenty-four 
hours. 

Hyperesthesia. — The  skin,  the  muscles,  the  joints,  can  hardly  bear 
the  slightest  touch  or  pressure,  and  sensitiveness  to  light  and  to  sound 
are  early  signs  of  meningeal  irritation.  Occasionally  the  sense  of  smell 
is  abnormally  acute. 

Nausea  and  Vomiting. — These  are  common  initial  symptoms  (80 
per  cent.).  Occasionally  the  vomiting  persists.  Early  vertigo  is 
frequent. 

Mental  Symptoms. — ^These  occur  early,  particularly  in  children,  and 
more  especially  in  tuberculous  meningitis.  The  patients  become 
peevish,  throw  their  toys  away,  are  capricious,  their  attention  varies. 
They  not  infrequently  have  mild  delirium  early,  especially  at  night, 
or  are  confused.  Very  young  children,  one  to  three  years,  often  show 
less  mental  involvement.  Older  patients  are  excited,  sleep  badly,  are 
restless,  mutter  in  their  sleep,  or  have  well-marked  delirium.    Later  a 


448  DISEASES  OF   THE  MENINGES 

semicoma  may  gradually  develop,  with  variations  in  intensity  from 
slight  confusion  to  a  confused  delirium.  Marked  ups  and  downs 
characterize  the  epidemic  forms,  but  some  degree  of  confusion  or  coma 
is  more  or  less  constant. 

Temperature. — The  patients  practically  always  show  a  rise  in  tem- 
perature. High  temperatures  (104°  to  106°  F.)  usually  characterize 
the  purulent  meningitides;  often  preceded  by  chill.  Such  tempera- 
tures may  remain  high,  or  not  infrequently  show  remissions.  The 
tuberculous  meningitides  usually  show  a  lower  curve. 

Motor  Irritation,  or  Paralytic  Phenomena. — Cramps  and  epileptiform 
convulsions  occur  more  often  in  the  early  stages  with  children  than 
with  adults.  They  sometimes  are  very  persistent.  Circumscribed 
spasms,  Jacksonian  in  type,  are  not  infrequent.  Occasionally  there 
are  choreic,  athetoid,  or  tremor  movements,  which  come  and  go,  or 
are  continuous. 

Muscular  rigidity  is  an  early  and  persistent  sign,  showing  early  in 
the  neck,  later  in  the  back,  with  opisthotonus,  or  stiff  lordosis  postures. 
These  muscular  rigidities  are  often  subject  to  considerable  fluctuation 
with  the  degree  of  internal  or  external  hydrocephalus.  Lumbar 
puncture  often  influences  them  greatly. 

Kernig's  sign  is  frequent,  and  is  based  upon  this  generalized  muscular 
rigidity  (reflex,  spasmodic  contraction  of  rectus  f emoris) . 

Paretic  signs,  monoplegia,  or  hemiplegia,  are  less  frequently  met 
with,  and  then  more  in  the  late  stages.  Such  indicate  localized 
purulent  processes,  with  abscess  formation  in  the  motor  area,  and  are 
usually  accompanied  by  convulsions.  Spinal  pareses  (paraplegia)  are 
rare. 

Speech  disturbances,  usually  cortical,  and  aphasia  are  occasionally 
observed,  more  particularly  in  tuberculous  meningitides. 

Cranial  Nerve  Signs. — Disturbances  of  the  cranial  nerves  are  among 
the  most  characteristic  signs  in  the  meningitides.  The  pupils  are 
often  unequal;  frequently  markedly  miotic  in  the  beginning,  they 
show  irregular  widening  later.  With  increasing  coma  they  usually 
widen,  and  react  sluggishly  or  not  at  all.  Convergence  reactions  are 
less  easily  tested,  but  also  show  slowness. 

Eye  palsies  are  frequent.  Mild  ptosis,  unilateral  or  bilateral,  diver- 
gent and  convergent  strabismus — the  abducens  is  particularly  prone 
to  disturbance — and  double  vision  is  often  present.  These  eye  palsies 
vary  from  hour  to  hour  in  extent,  and  in  intensity,  becoming  perma- 
nent in  the  long  protracted  cases  particularly.  Protrusion  of  the  eyeball 
is  a  rare  sign,  nystagmus  frequent. 

Papillitis  is  very  frequent,  and  early;  optic  atrophy  is  common  (18 
to  25  per  cent.).  Permanent  blindness,  however,  is  rare.  The  trigem- 
inus is  rarely  involved. 

Facial  palsies  are  very  frequent,  but  are  usually  incomplete,  vary 
from  day  to  day,  and  are  rarely  permanent. 

Hearing   is   frequently   affected.      Complicating   otitis,   and   laby- 


LEPTOMENINGITIS  449 

rinthitis  often  leave  these  patients  deaf.  It  is  a  frequent  complication 
in  the  second  and  third  week  of  the  disease. 

The  vagus  involvement  causes  pulse  and  respiratory  anomalies 
which  are  also  influenced  by  central  processes.  The  pulse  is  initially 
hastened  in  practically  all  forms,  and  usually  remains  rapid  in  the 
later  stages,  save  in  those  forms  of  less  acute  progress,  notably 
in  tuberculous  meningitis,  where  it  is  often  slow,  especially  after 
the  second  week  of  the  disease  (75  per  cent. — Heubner).  Marked 
irregularity  is  conspicuous. 

The  respiration  is  also  irregular  and  Cheyne-Stokes  type  is  frequent 
in  the  severe  purulent  and  tuberculous  types  toward  the  end. 

Reflexes. — The  tendon  reflexes  are  usually  somewhat  increased, 
particularly  in  the  early  stages,  but  may  be  missing  from  the  begin- 
ning (medullary  and  ventricle  pressure).  With  the  advance  of  the 
disease  they  may  be  missing.  Marked  variations  and  irregularities  are 
to  be  expected,  thus  lost  knee-jerks  may  be  associated  with  ankle- 
clonus  and  Babinski.  This  latter  is  a  very  frequent  early  sign  and  later 
disappears.  It  should  be  remembered  that  it  is  normally  present  in 
young  children  up  to  six  to  eight  j^ears  of  age,  and  hence  is  to  be 
neglected  in  diagnosis  in  young  children.^ 

Lumbar  Puncture. — This  is  of  primary  importance.  The  pressure 
is  initially  increased.  Later  this  is  not  marked  because  it  is  purulent. 
In  the  ordinary  purulent  meningitides  the  fluid  is  usually  cloudy, 
sometimes  only  microscopically  so,  again  markedly  purulent,  in  which 
the  specific  organisms  may  be  found  by  proper  methods.  In  tuber- 
culous meningitis  the  fluid  is  less  apt  to  be  cloudy,  especially  early, 
but  by  proper  technique  the  bacillus  is  found  (33  per  cent,  early  stages, 
50  per  cent,  with  pressure  signs,  100  per  cent,  in  paralytic  stages 
— Pfandler). 

Cytologically^  one  finds  that  in  purulent  meningitis  there  is  a  pre- 
ponderance of  poly  nuclear  leukocytes,  while  in  tuberculous  meningitis 
the  lymphocytes  are  increased.  This  is  not  an  absolute  rule  and  there 
are  variations  during  the  course  of  the  disease.  The  cytological  findings 
should  be  checked  up  with  the  clinical  ones. 

Irregular  Symptoms. — Herpes  is  not  infrequent  (75  per  cent.,  save 
in  young  children)  in  the  epidemic  form,  and  usually  appears  within 
the  first  week  (two  to  five  days).  It  is  most  frequent  about  the  nose, 
lips,  and  forehead.  It  does  not  persist  long  as  a  rule.  Other  skin 
eruptions  are  not  infrequent,  especially  the  rose-colored  spots  of  the  dip- 
lococcus  types.  Eruptions  resembling  measles,  scarlet  fever,  urticaria 
are  occasional,  while  erythemas  and  hemorrhagic  spots  are  rare. 

Gastric  disturbances  are  frequeni:,  especially  constipation.  Diarrhea 
may  be  an  initial  symptom  in  young  children. 

Tonsillar  and  pharyngeal  redness  and  soreness  are  not  infrequent 

^  See  Goppert  for  careful  study  of  reflexes;  Klin.  Jahr.,  xv,  523;  Berl.  klin.  Woch., 
1905,  21,  22. 

2  Schonbron,  Klin.  Vort.,  384;  Med.  Klin.,  1906,  p.  593. 
29 


450  DISEASES  OF  THE  MENINGES 

in  the  epidemic  types,  and  bronchitis  is  often  present  in  the  later 
stages. 

Course  and  Prognosis. — Whereas  most  of  the  types  of  leptomeningitis 
show  much  the  same  general  symptomatology,  it  is  more  particularly 
in  their  developmental  course  that  the  differences  appear.  These 
variations  may  be  viewed  as  fundamentally  due  to  specific  differences 
in  the  microorganisms  in  question,  although  it  may  be  said  that  in  a 
number  of  instances  the  only  differences  observed  by  the  best  clinical 
observers  have  been  those  of  the  microscopical  slide,  or  the  test-tube. 
Hence  it  may  be  stated  that  under  certain  circumstances  the  patholog- 
ical fact  that  the  patient  has  a  meningitis  governs  the  entire  picture 
and  all  forms  are  precisely  alike,  but  in  the  main  it  is  true  that  specific 
variations  exist,  and  should  be  sought  for  when  facing  any  particular 
case  of  leptomeningitis. 

In  general  fairly  sharp  distinctions  can  be  made  between  (1)  the 
infectious,  (2)  the  epidemic,  (3)  the  tuberculous,  and  (4)  the  serous 
forms. 

(1)  Infectious  Meningitis. — Here  the  initial  disturbance  in  the  ear, 
nose,  mastoid,  frontal  sinus,  fracture,  etc.,  precede,  and  its  symptoms 
often  hide  those  of  the  suppurative  meningitis  that  follows.  Here 
the  onset  is  usually  acute.  Headache,  local  or  diffuse,  is  early. 
The  temperature  usually  mounts  rapidly  to  104°  F.  or  105°  F.,  with 
initial  chill;  the  pulse  and  respiration  are  rapid.  There  is  photophobia 
and  hyperacusis  within  a  few  days.  The  mental  signs  come  on  soon. 
Confusion,  somnolence,  or  coma  are  frequent,  often  punctuated  by 
active  delirious  intervals.  Lumbar  puncture  usually  shows  a  purulent 
fluid.  The  tendon  reflexes  are  usually  increased  and  the  cranial  nerve 
signs  are  marked  within  the  first  week.  No  special  skin  eruptions  are 
noted.    Neither  tache  cerebrale  nor  dermographism  are  marked. 

With  irregular  septic  temperature,  increasing  coma  or  delirium, 
increasing  signs  of  local  pressure  or  destruction,  convulsions,  and 
paralyses  the  more  serious  cases  end  fatally  within  two  to  five  days  in 
children,  or  one  to  two  weeks  in  adults. 

Other  patients  show  less  grave  symptoms,  run  a  subactive  course 
and  recover  in  three  to  four  weeks,  but  in  general  the  prognosis  is 
unfavorable.  Those  that  get  well  probably  have  a  circumscribed 
process. 

(2)  Epidemic  Cerebrospinal  Meningitis. — Known  for  centuries,  first 
recognized  as  epidemic  in  1805  in  Switzerland,  in  1806  in  Massachu- 
setts, this  form  has  been  extensively  observed  the  world  over.^  Its 
epidemic  onset  is  usually  very  insidious,  and  spring  and  winter  are 
the  months  of  predilection  in  the  north  temperate  zones.  It  is  now 
endemic  in  large  cities.  Children  under  ten  are  most  prone  to  the 
infection.      The  exciting  agent  is  the  Meningococcus  intracellularis 

1  See  Report  of  State  Board  of  Health,  Massachusetts,  1898,  for  extensive  review  with 
literature  to  that  date.    Articles  of  Finklenburg,  loc,  cit.,  for  later  literature. 


EPIDEMIC  CEREBROSPINAL  MENINGITIS  451 

of  Weichselbaum.^  The  disease  is  contagious,  the  contagion  being 
possibly  carried  through  the  nasal  passages.  Abortive,  subacute, 
acute,  chronic,  and  fulminating  cases  are  recognized  with  every  pos- 
sible intermediary  type. 

The  malignant  or  fulminating  cases  may  end  fatally  in  twenty-four 
hours,  with  headache,  nausea,  vomiting,  delirium,  coma,  convulsions, 
stiff  neck,  high  temperature,  high  pulse,  Cheyne-Stokes  respiration 
and  death  from  acute  toxemia. 

Abortive  cases,  which  are  more  often  found  in  adults  than  in  children, 
show  rudimentary  meningeal  signs.  Headache,  backache,  nausea  and 
some  vomiting  with  slight  stiffness  of  the  neck  occur,  or  the  patients 
may  only  have  slight  vertigo,  malaise,  and  nausea,  and  keep  on  with 
their  work.  Fever  is  usual  and  occasionally  deafness  develops  in 
these  abortive  attacks. 

The  usual  subacute  or  acute  types  show  a  latent  period  averaging 
three  to  five  days  consisting  of  irregular  backache,  headache,  slight 
vertigo,  and  sweating,  with  signs  of  a  nasopharyngitis.  Then  a  chill 
with  slight  rise  in  temperature  accompanied  by  vomiting  indicates  the 
onset.  Children  become  restless,  cry,  and  are  very  irritable.  Usually 
within  twenty-four  hours  meningeal  signs  appear.  Stiff  neck,  headache, 
mental  confusion  are  present;  not  infrequently  convulsions  appear  in 
children.  Photophobia,  hyperacusis,  excessive  sensitiveness  to  pressure, 
and  hypertonicity  then  show  themselves  with  Kernig's  sign  and  in- 
creased tendon  reflexes.  The  cranial  nerve  signs  then  develop.  Herpes 
labialis  is  frequent  from  the  second  to  the  fifth  day,  and  a  measly, 
macular  eruption  (spotted  fever)  may  appear.  Other  skin  eruptions 
develop  in  the  first  week.  There  is  usually  a  leukocytosis,  and  the 
cerebrospinal  fluid  shows  a  characteristic  picture. 

The  coma  continues,  shows  considerable  variation  in  intensity,  and 
the  patient  gradually  improves,  or  shows  increasing  signs  of  pressure, 
convulsions,  palsies,  and  dies  within  seven  to  fourteen  days,  after 
ineffectual  attempts  at  maintaining  nourishment. 

Other  patients  show  a  much  more  chronic  course,  lasting  weeks  or 
months,  with  considerable  variation  in  the  intensity  of  the  headache, 
coma,  or  delirium,  with  eye  palsies,  mono-  or  hemiplegia,  contractures, 
constant  emaciation,  and  variable  temperature  until  death  takes  place, 
often  with  increasing  size  of  the  head — hydrocephalus  and  signs  of 
increased  cerebral  pressure. 

In  other  more  favorable  cases,  periods  of  clearness  or  of  betterment 
become  longer  and  more  pronounced,  intermingled  with  the  severer 
symptoms  already  outlined.  The  variability  in  clinical  course  is 
enormous,  but  the  prognosis,  up  to  the  time  of  the  introduction  of 
the  Flexner  serum,  was  distinctly  unfavorable,  the  mortality  ranging 
from  50  to  80  per  cent.  Apparently  mild  cases  often  die  and  some 
extremely  ill  children  recover.    Recovery  with  defect,  either  deafness, 

1  Fortschritte  d.  Med.,  1877,  p.  622. 


452  DISEASES  OF  THE  MENINGES 

blindness,  cranial  nerve  palsies,  monoplegias,  hemiplegias,  or  mental 
defects,  are  not  infrequent. 

Treatment. — Since  a  specific  antimeningitic  serum  has  been  elabo- 
rated by  Flexner^  the  prognosis  has  been  much  improved.  The  mor- 
tality has  fallen  to  25  per  cent,  in  some  of  the  recent  epidemics.^  The 
best  results  are  obtained  in  children  of  from  five  to  ten  years. 

The  effects  of  serum  treatment  are  often  seen  very  soon — twenty- 
four  hours  after  injection.  The  Kernig  sign  and  stiffness  of  the  neck 
persist,  however.  The  attenuation  of  the  symptoms  is  very  marked 
in  many  of  the  cases,  as  well  as  shortening  of  the  disease.  Lysis  is 
the  usual  mode  of  recovery  of  non-serum-treated  cases.  Crises  occur 
much  more  often  in  serum-treated  cases  (25  per  cent.).  Furthermore, 
the  permanent  sequelae  of  the  disease  are  markedly  reduced  by  the 
serum  treatment. 

The  general  treatment  will  be  considered  with  that  of  the  other 
forms  of  meningitis. 

(3)  Tuberculous  Meningitis. — This  form  was  first  isolated  about  1830 
(Rilliez  et  Barthez  and  Robert  Whytte),  although  one  can  see  its 
chief  features  in  the  Nosologie  of  Sauvages,  written  in  1763.  In  this 
form  the  onset  is  characteristically  subacute  or  chronic,  one  to  two 
weeks,  exceptionally  months,  and  is  almost  invariably  secondary  to 
tuberculosis  in  other  organs.  General  symptoms,  such  as  loss  of 
appetite,  irritability,  loss  of  flesh,  general  malaise,  with  loss  of  desire 
to  play,  fretfulness  and  ready  fatigability  are  the  precursors.  Head- 
ache and  dizziness,  with  irregular  fever  movement  is  then  observed, 
and  occasional  dreamy  states,  during  which  the  patient's  manner  is 
peculiar. 

Then  gradually,  or  suddenly,  the  patient's  manner  becomes  much 
changed;  mild  delirium  or  coma  develops,  and  convulsions  appear. 
The  patients  lie  in  bed,  are  restless,  rolling  from  side  to  side  with  sharp 
cries  or  whimpers,  and  frequent  placing  of  the  hand  to  the  head. 
Passive  motions  of  the  head  forward  invariably  bring  out  resistance 
and  pain.  The  sensitiveness  of  the  skin  to  pressure  or  touch  is  marked, 
and  hypertonus  with  muscular  twitchings  and  Kernig's  sign  are 
present. 

The  temperature  ranges  from  102°  F.  to  104"  F.,  and  is  usually  less 
marked  than  in  the  suppurative  or  epidemic  types.  Lumbar  puncture 
in  the  first  week  usually  gives  a  clear  fluid  under  pressure,  with  excep- 
tionally the  tubercle  bacillus  or  pus  elements. 

The  cranial  nerve  signs  may  then  develop  in  the  second  to  third 
week,  and  show  more  ups  and  downs  than  is  usually  present  in  the 
other  types.  Localized  pressure  signs  with  hemiplegias  or  monoplegias 
then  develop  and,  not  infrequently,  the  previously  increased  tendon 
reflexes  diminish  or  are  lost. 

The  patients  gradually  get  worse,  convulsions  are  frequent,  and 

1  Jour.  Exp.  Med.,  1907. 

2  Flexner,  International  Clinics,  1909;  Jour.  Amer.  Med.  Assoc,  October  30,  1909. 


SEROUS  MENINGITIS  453 

rigidity  is  followed  by  flaccidity,  and  the  patient  dies  with  signs  of 
intense  exhanstion,  sometimes  with  agonal  rise  of  temperature  imme- 
diately preceding.  The  whole  attack  may  terminate  within  a  compara- 
tively short  time,  two  to  three  weeks,  or  may  run  for  months. 

Atypical  forms  are  especially  frequent  in  adults  such  as  apoplecti- 
form onsets,  with  aphasia,  monoplegia,  or  hemiplegia.  The  picture 
may  be  that  of  a  toxic  delirium.    The  prognosis  is  bad. 

(4)  Serous  Meningitis. — Dietl,  in  1846,  first  isolated  this  type,  which 
is  of  uncertain  origin,  and  characterized  by  varying  grades  of  edematous 
exudate  with  infiltration  of  round  cells.  The  symptoms  are  usually 
those  of  a  mild  meningitis.  Headache,  stiff  neck,  marked  sensitive- 
ness are  constant,  whereas  convulsions  and  signs  of  pressure  are  less 
frequent.  Again  the  disorder  may  be  acute  and  very  severe,  with  high 
temperature  and  signs  of  cranial  nerve  involvement.  The  usual 
course  is  less  stormy.  Papillitis  is  a  frequent  symptom,  and  should 
be  borne  in  mind  in  those  patients  in  whom  the  serous  exudate  is 
more  or  less  circumscribed,  giving  the  general  impression  of  a  brain 
tumor.  Lumbar  puncture  shows  increase  in  pressure  with  some 
lymphocytes. 

In  the  serous  meningitis  of  alcoholic  origin  (wet  brain,  meningo- 
encephalitis), there  is  usually  a  busy  delirium,  excessive  hyperesthesia, 
with  marked  twitching  of  the  muscles. 

Syijhilitic  Meningitis. — See  Syphilis  of  the  Brain. 

Differential  Diagnosis, — The  chief  disorders  that  may  be  confounded 
with  the  meningitides,  especially  in  the  beginning  of  the  disorder,  or 
in  mild  cases,  are  as  follows:  Internal  pachymeningitis,  which  rarely 
gives  temperature  or  lumbar  puncture  findings.  Encephalitis,  and 
encephalomyelitis:  in  the  former  localizing  symptoms  develop  early, 
and  the  spinal  fluid  is  clear;  in  the  latter  flaccid  palsies  rapidly 
develop,  and  the  fluid  is  not  purulent.  Brain  abscess  may  be  com- 
plicated by  meningitis,  or  localized,  in  which  latter  case  the  localizing 
symptoms,  septic  course,  and  clear  spinal  fluid  are  of  aid. 

Infectious  sinus  thrombosis  may  resemble  meningitis  very  closely. 
There  is  a  greater  tendency  for  the  lower  cranial  nerves  to  be  involved, 
particularly  the  vagus,  hypoglossal  and  spinal  accessory.  The  spinal 
fluid  is  usually  clear. 

Delirium  tremens  shows  an  active  occupation  delirium,  optic  hallu- 
cinations, marked  fine  tremors  and  little  temperature.  Hysteria  rarely 
shows  temperature,  and  many  of  the  organic  signs  described  by  earlier 
authors  as  found  in  hysteria  are  better  referred  to  as  organic  brain 
disease  with  hysterical  epiphenomena. 

Treatment. — This  must  first  be  prophylactic,  and  involves  general 
hygienic  precautions  in  tuberculosis;  prompt  aural  treatment  in 
otitis,  mastoiditis;  surgical  asepsis  of  wounds  in  all  head  operations. 
The  isolation  of  the  epidemic  types  is  advisable,  and  nasal  antisepsis 
imperative.  The  active  treatment  is  surgical  for  most  of  these  forms, 
especially  if  the  symptoms  show  early  signs  of  being  circumscribed, 


454  DISEASES  OF  THE  MENINGES 

or  when  it  seems  possible  to  get  at  an  initial  focus  of  infection,  as  in 
the  various  septic  infectious  forms — ear  disease,  etc. 

In  diffuse  general  meningitis  the  responsibilities  are  great,  and  it 
is  difficult  to  decide  in  the  individual  cases. 

Epidemic  types  are  best  treated  by  serum.  Surgical  treatment  of 
tuberculous  meningitis  has  not  yet  met  with  sufiicient  success  to 
warrant  its  advocacy. 

Lumbar  puncture  has  given  very  brilliant  results  in  some  patients, 
in  others  it  has  been  of  little  service.  With  the  development  of  acute 
hydrocephalus  it  is  indicated,  and  it  is  in  general  a  harmless  procedure. 
The  punctures  may  be  made  frequently,  and  25  to  40  c.c.  of  fluid 
removed.    The  amount  removed  should  be  controlled  by  the  pressure. 

Of  the  serums,  the  Kolle-Wassermann  in  septic  cases  has  given  good 
results.    Flexner's  serum  has  been  mentioned. 

General  treatment  consists  in  keeping  the  patient  quiet.  Bromides, 
chloral,  veronal  and  other  mild  hypnotics  are  of  service.  Hypodermic 
use  of  morphin  or  hyoscin  may  be  necessary,  but  other  things  being 
equal,  is  undesirable.  The  continuous  hot  bath  is  especially  valuable 
in  delirious  patients. 

In  the  beginning  a  prompt  use  of  calomel  is  called  for:  The  room 
should  be  darkened  and  as  quiet  as  possible.  Pain  may  be  controlled 
as  far  as  possible  by  analgesics,  and  local  counter-irritants. 

Particular  attention  should  be  given  to  the  nourishment  and  rest 
of  the  patient.  Continuous  fussing  and  overnursing  is  to  be  avoided 
in  these  cases.  There  are  no  specific  remedies.  Urotropin  in  large 
doses  may  be  tried,  as  it  is  partly  broken  down  into  formaldehyde  in 
the  cerebrospinal  fluid.    The  silver  colloidal  salts  are  disappointing. 

In  the  long,  tedious  convalescence  of  many  of  these  little  patients, 
one's  ingenuity  is  taxed  to  stimulate  the  appetite,  provide  the  proper 
amount  of  outdoor  play,  and  to  strengthen  the  paretic  or  paralyzed 
muscles. 

2.  Chronic  Leptomeningitis. — This  usually  is  a  secondary  affection  in 
paresis,  senile  dementia,  chronic  lead  poisoning,  and  chronic  pachymen- 
ingitis; it  is  rare  as  a  primary  affection  save  as  syphilitic  or  tuberculous. 

Chronic  tuberculous  leptomeningitis  is  usually  of  the  convexity, 
usually  anterior,  involving  the  frontal  and  motor  areas.  It  behaves 
as  a  low,  mild-grade  meningitis,  often  with  intermittent  circumscribed 
symptoms,  simulating  those  of  a  brain  tumor. 

Chronic  traumatic  cases  often  show  vertigo,  pains,  epileptiform 
convulsions,  aphasias,  nausea  and  vomiting,  and  gradual  mental 
involvement.  These  signs  should  stand  in  direct  relationship  to  the 
injury,  and  should  not  be  complicated  by  signs  originating  at  a  dis- 
tance from  the  site  of  the  injury  or  from  other  causes  (Argyll-Robertson 
from  syphilis,  for  instance). 

Chronic  meningeal  inflammations  are  occasionally  found  in  children, 
giving  the  signs  of  a  posterior  basilar  meningitis.  They  are  often  com- 
plicated with  hydrocephalus.  Opisthotonus  is  frequent.  Many  are 
due  to  syphilis,  as  the  Wassermann  tests  show. 


HYDROCEPHALUS 


455 


Hydrocephalus. — An  accumulation  of  fluid  within  the  cranial 
cavities  takes  place  in  a  variety  of  affections.  It  is  invariable  in  the 
acute  meningitides,  in  greater  or  lesser  degree;  in  tuberculous  menin- 
gitis it  is  often  extreme  and  comes  on  with  great  rapidity — acute 
hydrocephalus  being  practically  synonymous  with  a  tuberculous 
basilar  meningitis. 

External  hydrocephalus  is  often  synonymous  with  serous  meningitis; 
as  an  entity  it  is  infrequent. 


Fig.  229. 


-Showing  marked  hydrocephalus.     Clinically  the  patient  showed  epileptic 
attacks  and  was  markedly  feeble-minded.     (Munson.) 


Hydrocephalus  is  a  very  frequent  complication  in  tumors  of  the 
brain,  particularly  in  tumors  of  the  third  ventricle,  the  pineal,  the 
corpora  quadrigemina,  and  those  causing  pressure  upon  the  aqueduct. 
These  conditions  are  discussed  under  Brain  Tumor.  Ventricular  hydro- 
cephalus often  results  from  such  structural  anamolies  as  cephalocele, 
and  spina  bifida. 

In  children  one  may  find  a  primary  chronic  hydrocephalus  of  as  yet 
unsettled  pathology;  but  there  is  usually  a  chronic  ependymitis  present. 


456  DISEASES  OF  THE  MENINGES 

One  to  five  pints,  even  gallons,  of  fluid  may  accumulate.  The  fluid 
is  clear,  slightly  alkaline  with  sp.  gr.  0.1005,  containing  the  earthy 
chlorides,  albumin,  phosphates,  and  occasionally  a  sugar-reducing  sub- 
stance. The  presence  of  high  percentages  of  albumin  and  many 
cellular  elements  indicates  a  more  active  inflammation.  As  a  result 
of  the  pressure  tlje  ventricles  widen,  the  septum  lucidum  is  displaced, 
the  cortex  thins,  the  infundibulum  dilates,  the  optic  chiasm  is  pressed 
upon.  Extreme  distention  may  convert  the  pallium  into  an  enormous 
cyst,  with  the  smallest  vestige  of  a  cortex.  The  thalamus  may  be 
flattened,  even  the  pons. 

The  cranial  bones  may  become  separated.  The  head  enlarges, 
usually  symmetrically,  at  the  vault  and  at  the  base.  In  older  children, 
however,  hydrocephalus  may  exist  without  these  changes  in  the 
position  of  the  cranial  bones  taking  place.  The  average  cranial  cir- 
cumference at  birth  is  fourteen  inches;  at  one  year  eighteen  to  nine- 
teen inches.  In  hydrocephalic  heads  this  is  increased.  As  a  rule  the 
congenital  types  show  the  largest  heads.  These  patients  are  rarely 
born  alive,  or  they  live  a  short  time  only. 

Symptoms. — Two  to  three  or  more  months  after  birth  it  is  noted 
that  the  child's  head  is  increasing  in  size  with  more  than  the  usual 
rapidity.  Somnolence  and  lethargy  are  frequent;  the  child  may  not 
be  able  to  hold  the  head  up.  A  whiny,  peevish  irritability,  with 
frequent  sharp  cries  is  the  rule.  With  fairly  rapidly  increasing 
internal  pressure  the  coma  is  marked,  showing  great  variability, 
however;  vomiting  is  frequent,  the  hearing  is  afi^ected,  also  the  sight, 
disks,  spastic  extremities,  with  usually  symmetrically  increased 
reflexes.  The  pupils  are  usually  contracted  and  sluggish  to  light  early. 
With  severe  grades  of  pressure,  there  may  be  extreme  dilatation. 
Convulsions  appear  and  death  results  in  from  three  to  six  months, 
with  signs  of  emaciation. 

In  the  cases  of  more  gradual  increase  of  intracranial  pressure  a 
marked  degree  of  accommodation  takes  place.  The  symptoms  are 
those  of  dulness  or  stupidity,  the  children  are  usually  less  bright,  the 
choked  disk  may  be  very  little  marked,  or  may  be  excessive  if  the 
bones  have  not  permitted  distention,  and  may  be  followed  by  atrophies; 
but  many  cases  recover  with  only  a  slight  degree  of  mental  reduction 
— weak-mindedness  or  only  stupidity.  There  is  a  proverbial  cheeri- 
ness  in  these  patients.  Some  few  make  total  recoveries,  and  may 
show  brilliant  mental  capacities. 

In  the  hydrocephalus  of  brain  tumor  in  adults,  with  its  up-and-down 
course,  somnolence  and  headache,  choked  disk,  dilated  pupils,  some- 
what inactive  to  light,  are  characteristic. 

A  cracked-pot  percussion  note  is  often  very  characteristic  in  the 
young  and  also  in  many  adults. 

Therapy. — Many  cases  are  hopeless  from  the  onset.  A  Wassermann 
test  should  always  be  made,  both  of  the  blood  and  cerebrospinal 
fluid,  for  the  double  purpose  of  determining  syphilis  or  other  inflam- 


HYDROCEPHALUS  457 

matory  disorder.  Many  cases  of  ependymitis  are  syphilitic  in  origin. 
For  these  prompt  mercurial  treatment  is  indicated;  salvarsan  or  enesol 
are  useful.  Inunctions  are  also  valuable  in  the  less  rapidly  advancing 
cases.    lodin  therapy  is  advantageously  combined. 

Cerebral  irritative  phenomena  need  hot  baths  and  bromides. 

Tapping  is  frequently  of  service,  but  not  always.  Lumbar  puncture 
is  not  practicable  to  relieve  pressure,  as  frequently  the  aqueduct  of 
Sylvius  is  blocked;  a  dry  lumbar  tap  is  of  diagnostic  service. 


CHAPTER   XII. 
DISEASES  OF  THE  BRAIN. 

ENCEPHALITIS— ABSCESS    OF   THE   BRAIN. 

Encephalitis  refers  to  an  inflammation  of  the  brain  substance. 
There  are  a  number  of  forms,  but  the  term  is  here  restricted  to  the 
more  acute  processes,  due  to  bacterial  or  toxic  action.  Primary  idio- 
pathic encephahtis  does  not  exist.  General  paresis  is  a  diffuse  enceph- 
alitis, with  exudations  and  proliferation  of  new  glia  and  bloodvessels; 
multiple  sclerosis  is  a  type  of  disseminated  encephalitis;  cerebral 
gummata  may  be  spoken  of  as  localized  encephalitis,  etc.,  but  the 
discussion  is  here  limited  more  particularly  to  acute  exudative  in- 
flammations of  the  brain  substance  proper.  Abscess  is  a  frequent 
secondary  result. 

Historical. — The  encephalitides  were  for  years  included  with  the 
meningitides,  possibly  under  the  term  phrenitis,  which  to  the  ancients 
meant  any  excitement,  accompanied  by  fever.  In  Hippocrates  the 
typical  description  of  phrenitis,  however,  is  a  typhoid  delirium. 
Traumatic  encephalitides  were  known  to  this  author. 

Just  how  long  this  conglomeration  of  cerebral  affections  remained 
an  entity  in  nosology  cannot  be  determined.  Separation  of  types  is 
going  on  at  the  present  time,  and  it  must  be  recognized  that  a  hetero- 
geneous group  at  best  must,  for  the  present,  be  included  under  the 
head  of  encephalitis,  a  term  used  as  early  as  1554  by  Actuarius. 

Leaving  aside  the  older  works,  the  history  of  encephalitis  practically 
begins  with  the  works  of  Rostan  (1820),  Bouiflard  (1826),  Lallemand 
(1830),  and  Fuchs  (1835),  in  which  processes  of  softening,  of  an 
inflammatory  or  non-inflammatory  character  are  commencing  to  be 
recognized.  Cruveilhier,  in  1829,  distinguished  an  apoplectic  soften- 
ing, suppurative  softening,  and  softening  with  disorganization  without 
pus  or  inflammation.  Virehow's  (1846)  work  on  thrombosis  and 
embolism  threw  much  light  on  the  subject,  while  the  studies  of  Durand 
Fardel  (1849),  Haye  (1868),  and  Huguenin  (1876)  commenced  to 
give  a  modern  touch.  Then  Wernicke  (1881)  described  a  toxic  form, 
chiefly  alcoholic,  and  Striimpell  (1884)  opened  up  the  large  study 
of  the  infective  types.  Finally,  the  modern  study  of  epidemic  polio- 
myelitis, by  Medin  and  Wickmann,  the  recognition  of  syphilitic  types 
by  Freud  and  Plant,  and  the  work  of  Councilmann  and  his  students 
have  served  to  widen  out  the  conception  of  the  infectious  types  of 
Striimpell.  Recent  monographs  of  value  are  by  Oppenheim  and 
Cassirer,  Nothnagel  (1907),  and  Southard  (Osier)  ^ 

1  Modern  Medicine,  1910. 


ENCEPHALITIS  459 

Etiology. — Infections  and  intoxications  are  many  and  various. 
Among  the  former  are  found  the  organisms  of  influenza,  streptococcus, 
typhoid,  Diplococcus  meningitidis,  pneumonia,  scarlet  fever,  measles, 
tuberculosis,  syphilis,  trypanosomiasis,  malaria,  rabies,  poliomyelitis, 
etc.  The  commonest  acute  forms  are  due  to  the  influenza  bacillus, 
pyogenic  organisms  (external  and  internal  infections),  including  mas- 
toid, etc.,  poliomyelitis;  among  the  more  common  chronic  types 
are  tuberculosis  and  syphilis,  which  latter,  as  Plaut^  has  well  shown, 
is  responsible  for  many  of  the  infantile  cerebral  palsies.  As  for  the 
intoxications,  alcohol  plays  the  chief  role. 

Acute  Encephalitis. — Symptoms. — As  so  much  depends  upon  the  seat 
of  the  inflammatory  process,  it  is  natural  that  great  variations  in  the 
clinical  picture  should  be  expected.  The  type  of  infecting  agent  also 
introduces  a  variant.  The  infecting  agent  of  a  polio-encephalo-myelitis, 
is  different  from  that  of  a  syphilitic  encephalitis.  Although  similar 
structures  may  be  involved  and  in  a  manner  very  closely  related  patho- 
logically, yet  nevertheless  the  mode  of  development  will  be  different. 
For  this  reason  some  of  the  more  pronounced  types  will  receive 
separate  consideration.  Any  attempt  to  generalize  on  the  whole 
group  results  in  a  descriptive  monstrosity  untrue  to  any  clinical 
type. 

1.  Influenza  Encephalitis. — The  work  of  Wickmann  and  others  has 
shown  that  great  care  must  be  exercised  in  not  confusing  this  wuth 
poliomyelitis.  In  the  true  influenzal  type,  the  disorder  is  apt  to 
develop  in  young  adults  some  days,  or  even  weeks,  after  an  acute 
influenza.  Oppenheim  includes  other  infectious  types  here.  The 
"  cold  in  the  head"  seems  to  be  clearing  up,  when  most  intense  headache 
— often  in  the  occiput — nausea,  vomiting,  apathy,  or  drowsiness  com- 
mences to  indicate  something  more  than  the  usual  depression  of  an 
influenzal  attack.  Sometimes  there  is  a  rigor.  Confusion  and  mild 
stupor  are  frequent,  the  patient  being  aroused  with  some  difficulty 
after  a  few  days.  Symptoms  of  meningeal  irritation  are  not  prominent, 
and  a  clear  cerebrospinal  fluid  will  separate  this  condition  from  the 
closely  related  picture  of  cerebrospinal  meningitis.  There  is  usually 
much  febrile  irregularity.  The  pulse  is  very  variable,  being  not  infre- 
quently slow. 

Focal  symptoms  develop  irregularly.  If  the  pyramidal  region  is 
involved  there  is  premonitory  weakness  of  the  limbs,  then  paresis, 
then  paralysis.  Convulsive  seizures  may  be  present.  The  extent  may 
be  that  of  a  monoplegia  only,  or  a  hemiplegia,  which  may  not  show 
in  the  coma.  Various  aphasias  may  occur,  pseudobulbar  palsies,  or 
occulomotor  palsies.  Sensory  anomalies  are  frequent,  but  are  difficult 
to  detect  because  of  the  mental  state. 

Many  patients  recover  absolutely,  others  are  left  with  slight  motor 
defects,  monoplegias,  or  severe  hemiplegias.    In  a  few  abscess  may 

1  Nervous  and  Mental  Disease  Monograph  Series,  1911. 


460  DISEASES  OF  THE  BRAIN 

develop.    In  others  there  is  a  complicating  ependymitis  with  hydro- 
cephalus, and  frequentl}^  there  are  psychotic  complications. 

2.  Polioencephalomyeliiis  (Striimpell's  acute  encephalitis  of  chil- 
dren in  part) . — This  has  been  mentioned  under  Anterior  Poliomyelitis. 
The  disorder  shows  the  usual  acute  onset  of  this  disease.  The  cerebro- 
spinal fluid  shows  the  characteristic  bodies  described  by  Hough  and 
Lafora;  the  Wassermann  reaction  of  the  cerebrospinal  fluid  should  be 
negative.  The  residuals  here  may  be  purely  in  the  cerebrum,  the 
midbrain,  pons,  medulla,  and  spinal  cord  escaping  entirely.  Various 
forms  of  infantile  palsy  (so-called  Little's  disease),  idiocy,  imbecility, 
monoplegia,  etc.,  are  to  be  encountered.  Epileptiform  convulsions 
are  an  infrequent  residual  of  a  motor  zone  focus.  The  prognosis  is 
usually  good.  Many  patients  recover  entirely,  or  with  mild  residuals. 
As  a  rule  there  are  pontine,  bulbar,  or  spinal  complications. 

3.  Polioencephalitis  Hemorrhagica  Superior. — Wernicke  called  atten- 
tion to  a  special  form  of  diffuse  encephalitis,  with  pronounced  involve- 
ment of  the  midbrain  (ophthalmoplegias).  In  one  sense  this  is  not  a 
true  inflammatory  reaction.  In  the  alcoholic  cases  the  course  of  the 
affection  resembles  that  of  the  Korsakow  syndrome,  with  pronounced 
eye  palsies.  There  is  usually  an  initial  delirium,  sometimes  appearing 
later.  Headache,  nausea,  and  vomiting  precede  the  development  of 
an  irregular  type  of  ophthalmoplegia,  apparently  nuclear,  possibly 
neuritic.  Ptosis,  nystagmus,  irregular  palsies,  optic  neuritis  are 
frequent.  The  gait  is  cerebellar;  the  speech  is  slurring;  the  mental 
disorientation  for  time  and  place  and  confabulation  are  marked. 
Somnolence  and  loss  of  sensory  functions  are  also  frequent,  and  point 
to  the  involvement  of  the  thalamus.  There  is  no  fever,  no  leukocy- 
tosis, and  a  doubtful  cerebrospinal  fluid.  The  pulse  is  usually  rapid — 
80  to  120 — tachycardia.  The  nerve  trunks  and  muscles  are  usually 
tender.  Many  of  these  patients  clear  up  to  a  marked  degree,  but  there 
is  usually  a  residual  mental  defect,  showing  in  a  lack  of  initiative  and 
mild  demented  states. 

The  lesions  are  those  of  a  diffuse  neuritis,  with  hemorrhagic  foci. 

A  non-alcoholic  variety  has  been  described,  in  which  somnolence 
is  more  marked  than  delirium.  There  is  ptosis,  complete  ophthalmo- 
plegia, with  vertigo  and  ataxia,  disorientation  and  frequently  confabu- 
lation. 

Occasionally  poisoning  by  mussels,  by  fish,  bromides  or  from  rabies 
shows  similar  clinical  pictures. 

4.  Pyogenic  Types. — Here  a  multiplicity  of  affections  is  to  be  reck- 
oned with.  Age  is  no  bar.  Traumatism  bulks  high  in  the  percentage; 
also  ear  disease.  The  onset  is  usually  acute,  particularly  in  the 
younger  patients.  Trauma,  or  middle  ear,  labyrinthine,  sinus  or 
other  pyogenic  infection  is  followed  by  malaise,  headache,  nausea, 
and  vomiting  with  advancing  stupor,  or  convulsions  and  increasing 
coma.  Great  restlessness,  with  developing  delirium,  usually  develops 
within  from  three  days  to  two  weeks.    Leukocytosis  is  usually  present. 


ENCEPHALITIS  461 

Lumbar  puncture  is  usually  negative,  except  when  meningeal  symptoms 
are  also  present. 

Local  topical  signs  are  frequent.  These  are  monoplegia,  hemiplegia, 
epileptiform  convulsions,  cranial  nerve  palsies.  If  the  delirium  clears 
up,  one  may  find  aphasias,  pseudobulbar  palsies,  various  midbrain 
lesions,  hemianopsias,  and  bulbar  palsies. 

Many  patients  die  in  coma.  Others  recover  with  marked  mental 
defect  (cerebral  atrophies  of  childhood,  idiocy,  imbecility,  debility). 
Li  others  the  general  signs  of  brain  abscess  (g.  v.)  become  apparent. 
Again  others  clear  up  with  hemiplegias,  diplegias  (so-called  Little's 
disease).  Finally  others  recover  entirely,  or  show  small  focal  residual 
lesions. 

Diagnosis. — It  is  impossible  with  present  methods  to  clearly  dis- 
tinguish the  various  encephalitides.  Many  are  not  diagnosed  during 
life,  the  course  being  so  rapid,  and  facilities  for  laboratory  research 
limited.  Oppenheim  has  well  said  that  at  the  present  time  (1911) 
any  attempt  at  presenting  the  problem  is  only  patchwork. 

Wernicke's  type  is  characteristic.  It  is,  however,  frequently  reserved 
for  the  psychiatrist  to  make  the  diagnosis  as  the  mental  symptoms  so 
mask  the  physical  signs.  Ophthalmoplegia,  neuritis,  disorientation 
for  time  and  place,  with  confabulation,  progressing  to  delirium  or 
coma,  the  almost  typical  facies,  closely  resembling  the  Korsakow  facies, 
tachycardia  and  no  temperature  are  the  leading  features.  Here  the 
entire  series  of  the  alcoholic  syndromes  must  be  reviewed.  (See 
Alcoholism.) 

Polioencehalomyelitis  is  to  be  suspected  during  an  epidemic. 
The  acute  onset,  frequently  with  gastro-intestinal  or  nasal  respira- 
tory predecessors;  the  frequency  of  spinal  and  bulbar  involvements; 
usual  absence  of  signs  of  neuritis;  lost  knee-jerks,  spinal  fluid  findings 
are  suggestive. 

Meningitis  often  calls  for  diagnosis.  Here  lumbar  puncture  is  of 
great  ser^dce,  since  many  of  the  initial  clinical  signs  are  identical. 
Increased  pressure,  presence  of  cellular  elements  and  pus  are  present 
in  purulent  meningitis.  In  epidemic  cerebrospinal  meningitis  there 
is  the  characteristic  organism. 

Herpes  is  usually  absent  in  encephalitis;  the  neck  and  muscle 
hyperesthesia  usually  less.  Eye  muscle  palsies  are  often  absent 
in  the  pyogenic  types  of  encephalitis;  they  are  not  infrequent 
in  meningitis.  In  syphilitic  types  the  Wassermann  is.  of  great 
service. 

Therapy. — Practically  only  in  the  malarial  and  syphilitic  encephali- 
tides is  there  any  specific  therapy.  In  the  pyogenic  forms,  with  abscess, 
surgery  is  demanded.  The  poisonings  require  withdrawal.  Bromide ■ 
poisoning  is  not  to  be  overlooked. 

All  these  patients  require  bed  treatment  in  a  darkened  room.  The 
toxic  cases  should  have  hot  sheets  and  elimination  be  encouraged.  Ice- 
bags  to  the  head  give  comfort  and  counter-irritation;   they  do  not 


462  DISEASES  OF  THE  BRAIN 

affect  internal  temperatures.     Active  catharsis  is  desirable,  calomel 
and  salines  being  advisable. 

In  the  influenza  types,  salicylates  are  indicated.  Otherwise  the 
treatment  is  largely  symptomatic.  The  residual  symptoms  call  for 
their  special  therapy. 

ABSCESS    OF   THE   BRAIN. 

History. — Brain  abscesses  have  been  recorded  for  many  years: 
Cruveilhier's  and  Carswell's  illustrations  are  classic.  The  steps  that 
led  up  to  the  present-day  conception  of  brain  abscess  are  partly  out- 
lined in  the  section  on  Encephalitis.  The  history  of  these  two  groups 
is  almost  identical  up  to  the  appearance  of  Wernicke's  Lehrbuch  d. 
Gerhirnkrankheiten,  1883,  when  a  division  into  infectious  and  non- 
infectious abscesses  was  foreshadowed.  MacEwen's  (1893)  studies 
gave  a  great  impetus  to  the  understanding  of  the  ports  of  entrance, 
while  Korner's  (1902-1908)  studies  have  set  in  clear  relief  the  impor- 
tance of  aural  disease.  Recent  monographic  treatments  are  those  of 
Oppenheim  and  Cassirer  (1909),  Southard,  in  Osier's  Modern  Medicine 
(1912),  and  Brissaud  and  Soques,  in  Bouchard  and  Brissaud's  Traite 
de  Medicine  (1904),  ix. 

Etiology. — Cerebral  abscess  is  almost  exclusively  a  secondary  phenom- 
enon due  to  infection.  Many  organisms  have  been  found  in  cerebral 
abscess.  They  are  derived  from  many  sources;  external  trauma,  the 
venous  sinuses,  and  otitis  media  being  among  the  commonest.  Otitic 
infection  supplies  about  a  third,  the  usual  communication  being 
through  the  tegmen  of  the  antrum.  Metastatic  abscesses,  from  pyemia, 
tuberculosis,  osteomyelitis,  abscess  of  lung,  empyema,  etc.,  are  not 
infrequent.  Actinomycosis  and  oidium  infections  are  among  the 
curiosities.  Streptococcus  pyogenes  is  the  most  frequently  found 
bacterium. 

Symptoms. — There  is  always  some  antecedent  disorder,  although 
such  may  be  overlooked  or  silent,  the  acute  miliary  tuberculosis  of 
cardiac  disease  being  an  example.  The  symptoms  will  vary  greatly 
with  the  exciting  cause,  the  site,  and  the  size  of  the  abscess  or  abscesses. 
Certain  traumatic  abscesses  run  their  fatal  course  in  three  to  five  days, 
whereas  some  recorded  cases  have  persisted  for  years. 

There  are  enough  cases  described  to  show  that,  speaking  in  general 
terms,  one  may  recognize  (1)  a  stage  of  irritation,  (2)  a  stage  of  remis- 
sion or  latency,  and  (3)  a  stage  of  paralysis  or  of  acute  progression. 

1.  Primary  Stage. — In  the  initial  stages,  general  rather  than  localiz- 
ing signs  predominate.  The  patient  usually  has  an  acute  rise  in 
temperature;  it  is  frequently  slight,  insidious,  and  variable,  and  often 
absent  after  a  few  days.  The  pulse  is  often  slow.  Headache  is 
rarely  absent  but  is  very  variable  in  intensity.  It  is  most  frequently 
dull,  but  gives  a  sense  of  tension  in  the  head.  There  may  be  some 
vomiting. 


ABSCESS  OF   THE  BRAIN  463 

Such  a  tense  headache,  with  rise  in  temperature  and  slow  pulse, 
following  an  otitis,  after  the  discharge  may  have  ceased,  is  of  grave 
import.  The  pain  is  situated  usually  over  the  side  affected;  but  is  not 
infrequently  slight  or  absent  in  cerebellar  cases  {q.  v.)  or  here  may  be 
frontal  in  character.  Movements  are  apt  to  increase  the  headache. 
Anxiety,  insomnia,  restlessness  are  the  rule,  and  in  younger  patients, 
the  sleep  is  often  broken  by  loud  cries.  There  may  be  some  delirium 
or  stupor  in  these  initial  stages,  which  occupy  the  first  three  or  four 
days,  occasionally  a  week.  There  is  frequently  some  leukocytosis  in 
this  stage. 

2.  Stage  of  Remission  or  Latency. — A  period  of  depression  often  sets 
in  about  this  time.  The  headache  diminishes,  the  fever  diminishes, 
the  patients  are  fatigued,  somnolent,  indifferent.  The  pulse  is  apt  to 
remain  slow.  Percussion  at  this  time  may  reveal  tenderness.  The 
blood  count  is  variable,  with  a  tendency  to  polynuclear  increase. 
This  stage  shows  extraordinary  variation  in  different  cases,  sometimes 
persisting  weeks  or  months. 

In  this  stage  beginning  choked  disk  is  not  infrequent,  especially  if 
the  disorder  has  lasted  over  a  week  or  ten  days.  It  is  apt  to  first  appear 
homolateral  with  the  abscess.  There  are  indefinite  signs  of  a  cerebral 
affection;  variable  headache,  occasional  nausea,  vomiting,  fainting 
attacks,  or  convulsive  seizures. 

The  pupils  are  frequently  unequal,  if  there  is  pus,  and  the  more 
dilated  pupil  is  apt  to  be  on  the  side  of  the  abscess.  With  maximum 
equal  dilatation,  the  localization  is  difficult  from  the  pupils  alone, 
and  such  dilatation  argues  for  a  large  abscess.  With  increasing  size, 
immobility  appears.  Contracted  pupils  are  found,  in  a  few  instances, 
toward  the  end  in  severe  cases. 

3.  Stage  of  Advance. — After  a  period  averaging  from  one  to  three 
months  the  symptoms  of  an  advancing  cerebral  pressure  manifest 
themselves.  There  are  delirium,  often  epileptiform  convulsions,  all 
of  the  symptoms  of  a  generalized  meningitis,  with  death. 

Occasionally  encapsulation  occurs  with  no  symptoms  for  from  ten 
to  twenty-eight  years^  (Nauwerck). 

Course. — The  course  just  described  is,  statistically  speaking,  the 
most  frequent,  but  it  is  by  no  means  universal.  In  many  cases  there 
is  an  acute  progressive  course,  without  any  intermission  or  remission. 
This  may  also  follow  a  latent  period,  or  may  develop  acutely  after  a 
trauma,  or  following  a  suppurative  process  in  the  ear  or  nose.  In  others 
there  occurs  an  acute  progressive  course,  with  a  remission  which  is 
incomplete.    The  chronic  course  is  as  described. 

Forms. — The  most  marked  are  the  traumatic,  the  otitic,  the  rhino- 
genic,  and  metastatic.  In  the  traumatic  cases  the  abscess  is  usually 
near  the  site  of  the  trauma,  and  the  symptoms  of  the  abscess  formation 
are  apt  to  be  complicated  by  the  traumatic  incidents.    The  symptoms 

*  See  Oppenheim,  loc.  cit.,  for  literature  of  these  rare  eases. 


464  DISEASES  OF   THE  BRAIN 

of  a  leptomeningitis  develop  early,  whereas  those  of  a  purulent  destruc- 
tive nature  usually  require  from  eight  to  fourteen  days  (Bergmann). 
The  whole  development  is  usually  gradual,  and  the  stages  far  from 
being  sharply  set  off  one  from  another.  Headache,  fever,  vomiting, 
vertigo,  confused  delirium,  these  are  the  prodromal  signs.  The  focal 
lesions  occur  several  days  later,  either  as  an  epilepsy,  a  cortical  mono- 
plegia, a  speech  or  other  motor  or  sensory  defect. 

Traumatic  late  abscesses  are  also  known,  weeks,  months,  even  years 
go  by,  before  the  full  truth  is  known. 

Otitic  brain  abscesses  are  frequent,  yet  in  proportion  to  otitis  are 
rare  (Jansen,  7  in  5000  cases).  Here  the  original  disorder,  the  local- 
ization, and  the  complications  are  of  moment.  The  development  has 
already  been  sketched.  It  is  very  insidious  in  many  cases.  The 
localization  is  usually  either  temporosphenoidal  or  cerebellar.  The 
symptomatology  of  right-  and  left-sided  temporosphenoidal  abscesses 
or  tumors  offer  considerable  variations.  A  right-sided  abscess  may 
pursue  its  course  with  little  or  no  localizing  signs.  Left-sided  ones 
often  give  rise  to  aphasic  complexes.  Pressure  upon  the  pyramidal 
tracts  will  give  characteristic  hemiplegic  phenomena,  there  are  apt 
to  be  disturbances  of  smell,  direct  or  subjective,  olfactory,  or  uncinate 
fits  (Knapp,  loc.  cit.). 

Rhinogenic  abscesses  are  usually  located  frontally.  In  addition  to 
the  headache,  there  is  frequently  a  certain  degree  of  torpor,  often 
associated  with  a  tendency  to  joke  (Witzelzucht),  isolated  choked  disk, 
a  cerebellar  type  of  ataxia,  when  the  rubrocortical  fibers  are  implicated, 
isolated  tremor  of  the  hand,  and  olfactory  disturbances.  When  the 
abscess  reaches  farther  back,  motor  symptoms  occur. 

Diagnosis. — Many  differential  points  arise.  The  etiology  is  the  most 
important  factor  to  bear  in  mind.  Traumatic  purulent  meningitis, 
apoplexy,  late  apoplexy  (Bollinger),  pachymeningitis,  brain  tumor, 
tuberculous  meningitis,  syphilitic  meningitis,  hemorrhagic  encephalitis, 
meningitis  serosa,  functional  neurotic  headache  and  sinus  thrombosis 
are  the  chief  disorders  to  be  reviewed.  The  last  often  runs  a  very  simi- 
lar course,  and  is  often  associated  with  a  purulent  otitis,  as  a  funda- 
mental disorder.  Here  the  fever  is  apt  to  be  high  and  remittant,  even 
on  the  same  day,  rigors,  chills,  and  profuse  perspiration,  pulse  usually 
rapid  and  irregular,  convulsions  rare,  save  perhaps  in  children,  head- 
ache constant,  eye-grounds,  as  in  abscess,  usually  bilateral  neuritis, 
more  frequent  than  choked  disk.  Focal  symptoms  rare.  Physical  signs 
of  swollen  spleen,  and  metastatic  invasions  confirm  the  diagnosis  of 
thrombosis. 

A  purulent  meningitis  may  be  more  difficult  to  differentiate.  It  may 
be  caused  by  similar  factors ;  it  is  usually  more  acute  and  stormy  in  onset ; 
it  usually  runs  a  shorter  course,  shows  higher  and  more  continuous  fever, 
herpes,  the  pulse  usually  rapid,  irregular,  often  slow  in  beginning  as  in 
abscess,  initial  unrest,  irritability,  confusion,  delirium,  in  contrast  to  a 
heaviness  or  coma  in  abscess,  headache  constant,  vomiting  frequent. 


ABSCESS  OF   THE  BRAIN  465 

convulsions  common,  general  clonic  and  often  muscular  twitches,  the 
eye-grounds  are  less  often  positive,  meningeal  irritation  phenomena 
(Kernig,  spasticity,  clonus,  etc.)  common,  local  symptoms  more  con- 
fined to  cranial  nerves  and  basal  signs  rather  than  intracerebral  focal 
signs,  spinal  symptoms  frequent,  whereas  in  abscess  they  are  rare. 
Furthermore,  the  cerebrospinal  fluid  is  more  apt  to  contain  globulin 
and  show  a  lymphocytosis  or  even  pus. 

Prognosis. — When  left  alone,  cerebral  abscess  almost  invariably 
causes  death.    Calcification  or  external  discharge  occasionally  occurs. 

Treatment, — This  is  exclusively  prophylactic  in  nose,  throat,  and 
otitic  work,  and  surgical  after  the  development  of  the  abscess.  The 
results  of  operative  interference  are  highly  satisfactory.  The  statistics 
vary  with  each  new  series  of  cases.  In  the  hands  of  competent  surgeons 
the  risk  from  operation  is  nil.  The  temporosphenoidal  and  frontal 
abscesses  can  be  readily  reached  and  drained.^ 

1  Starr,  Med.  Record,  1906. 


30 


CHAPTER  XIII. 
DISEASES  OF  THE  BRAIN  (Continued). 

VASCULAR  DISTURBANCES— CEREBRAL  APOPLEXY. 

The  quantity  and  quality  (viscosity)  of  the  blood,  the  heart  action, 
and  the  size  and  activity  of  the  bloodvessels  are  the  chief  factors 
governing  the  general  blood  flow,  and  hence  that  of  the  brain.  Of  all 
the  bodily  structures,  the  brain  is  among  the  most  highly  vascularized, 
arguing  for  its  great  functional  activity.  Furthermore,  there  is  marked 
plasticity  evident  in  the  compensatory  regulations.  Not  only  is  there 
a  continuous  balance  maintained  between  different  vascular  systems, 
especially  between  those  larger  groups,  such  as  the  portal  system,  the 
vessels  of  the  skin,  the  muscles,  and  the  brain,  but  within  the  brain 
itself  different  balancing  reactions  are  constantly  going  on  between 
speech  areas,  visual  areas,  auditory  areas,  motor  systems,  etc.,  which 
are  thrown  into  use  for  instance  in  specialized  occupations  or  activities. 

The  anatomical  structures  used  in  regulating  these  compensating 
mechanisms  are  partly  localized  in  the  medulla  and  cord,  as  discussed 
in  the  chapters  on  the  Vegetative  Neurology  of  the  Vascular  Appara- 
tus, but  parts  are  within  the  vascular  apparatus  itself;  at  times  with- 
in the  walls,  again  within  the  circulating  blood  (viscosity  disturbance) . 

The  smaller  cerebral  vessels,  in  uniformity  with  the  softness  and 
plasticity  of  a  developing  organ,  have  less  rigid  walls,  hence  more  easily 
overfill  (congestion)  and  contract  (fainting),  are  more  liable  to  spon- 
taneous rupture,  and,  furthermore,  many  differ  in  that  they  have  few 
or  no  anastomosing  branches.  Thus  special  problems  are  connected 
with  the  cerebral  circulation.  The  avenues  of  blood  intake  are  through 
the  two  internal  carotids  and  the  two  vertebrals.  The  course  of  the 
left  internal  carotid  is  more  direct,  and  it  has  been  taught  that  thrombi 
are,  therefore,  more  common  on  the  left  side,  which  has  no  decisive 
facts  to  bear  out  the  a  'priori  hypothesis. 

The  vertebrals  unite  to  form  the  basilar,  which  bifurcates  into  the 
two  large  posterior  cerebral  vessels  which  supply  the  temporo-occipital 
lobes,  the  corpora  quadrigemina,  crura,  and  parts  of  the  optic  thalami. 
The  posterior  communicating  branches,  usually  small  and  symmetrical 
and  subject  to  great  variation,  pass  forward  to  join  the  internal  carotids, 
and  are  given  off  to  the  base  of  the  brain.  The  internal  carotids  form 
the  middle  cerebral  and  the  anterior  cerebral  arteries,  an  anterior 
communicating  branch  completing  the  circle  of  Willis. 

The  middle  cerebral  arteries  lie  in  the  Sylvian  fissure,  giving  off  many 


VASCULAR  DISTURBANCES 


467 


branches  to  the  lenticular  nucleus,  the  internal  capsule,  portions  of 
the  thalamus,  and  superficially  irradiate  the  Island  of  Reil,  Heschl's 
convolution,  and  much  of  the  lateral  aspect  of  the  brain. 

The  anterior  cerebral  arteries  supply  the  frontal  areas,  the  olfactory 
apparatus,  the  upper  margin  of  the  frontal,  parietal,  and  occipital 
lobes  on  their  mesial  aspects,  and  much  of  the  corpus  callosum. 

The  cerebellum  derives  its  supply  from  the  vertebral  and  basilar 
arteries. 

Partial  compensatory  balance  of  the  circulation  is  brought  about 
chiefly  through  the  circle  of  Willis.    This  circle,  as  well  as  the  branches 


Anterior  communicating 


Anterior  cerebral 


Middle  cerebral- 


Anterior  choroid 

Posterior  comviunicating 

Posterior  cerebral 

Sujyerior  cerebellar- 
Basilar 
Anterior  inferior  cerebellar 

Interior  auditorij 
Posterior  inferior  cerebellar- 
Vertebral 


Posterior  spinal 


Anterior  spinal 


Fig.  230. — Diagram  of  the  arteries  at  the  base  of  the  brain,  including  the  circle  of 
Willis.  J,  anteromedian  group  of  ganglionic  branches;  II,  posteromedian  group; 
III,  right  and  left  anterolateral  group;  IV,  right  and  left  posterolateral  group.    (Gray.) 

from  it,  shows  a  vast  number  of  anomalies,  and  these  in  part  determine 
many  anomalous  cerebral  disturbances,  possibly  related,  as  Blackburn, 
Windle,  Bullen,  and  others  have  shown,  to  faulty  cerebral  development, 
thereby  laying  the  anatomical  foundation  for  a  psychosis  or  some 
aberrant  vascular  disease.  Furthermore,  such  anomalies  are  of  great 
surgical  importance.^ 

The  chief  cerebral  arterial  disorders  arise  from  (a)  temporary 
vascular  instability  (shock  reactions,  cardiac  irregularities,  internal 
secretory  disturbances),    (b)   protracted  regressive  changes   (arterio- 


1  I.  W.  Blackburn,  Jour.  Comp.  Neur.,  1907,  vol.  xvii  and  xx,  1910,  185. 


468  DISEASES  OF   THE  BRAIN 

sclerosis,  with  or  without  mihary  aneurisms),  (c)  hemorrhage,  (d) 
thrombosis,  (e)  emboHsm. 

Venous  changes  are  less  frequent  than  arterial  ones.  Sinus  throm- 
boses from  infectious  processes  are  the  most  important. 

(a)  Vascular  Instability. — This  is  a  very  variable  factor  and  has 
been  discussed  under  the  sections  on  Vegetative  Neurology.  Vagotonia 
and  vagotropia  occur  in  the  cerebral  vessels  as  well  as  in  the  somatic 
vessels.  The  results  were  previously  grouped  under  the  concepts 
cerebral  anemia  and  cerebral  hyperemia.  Chronic  vagotonic  condi- 
tions are  known.    They  should  not  be  called  cerebral  congestions. 

Anemia  (Shock;  noci-association — Crile)  results  from  loss  of  blood, 
paracentesis,  surgical  handling  of  intestines,  cardiac  weakness,  or 
from  marked  vascular  instability,  often  of  emotional  origin.  The 
symptoms  are  faintness,  dizziness,  black  spots  before  the  eyes,  buzzing 
in  the  ears,  and  it  may  be  loss  of  consciousness  with  or  without  nausea 
and  vomiting.  There  may  be  partial  consciousness,  apathy,  or  semi- 
coma, with  contracted  pupils,  and  cold  and  clammy  skin,  occasionally 
loss  of  bladder  or  bowel  function. 

The  therapy  is  heat  and  bandaging  of  the  extremities,  the  horizontal 
position,  camphor,  caffein,  and  cardiac  stimulants,  alcohol  and  ether. 

Hyperemia. — Hyperemia  may  be  active  or  passive.  The  former  may 
result  from  vascular  instability,  or  is  not  infrequent  after  excessive 
eating  or  drinking.  Sudden  emptying  of  the  peripheral  and  somatic 
vessels  may  occasion  it.  It  is  frequent  in  certain  thyroidisms,  notably 
in  exophthalmic  goiter  and  in  natural  and  artificial  menopause  states, 
often  due  to  changes  in  the  internal  secretions.  (See  Vegetative 
Neurology.) 

The  symptoms  of  hyperemia  are  redness  and  congestion  of  the 
face  and  eyelids,  pounding  in  the  ears,  or  neck  or  head,  headache, 
confusion,  and  usually  contracted  pupils.  More  severe  attacks  may 
lead  to  acute  confusion,  rise  in  temperature,  and  the  general  picture  of 
an  acute  meningitis. 

Signs  of  congestion  of  the  face — flushing,  a  sense  of  fulness,  etc., 
must  not  be  taken  to  necessarily  mean  cerebral  congestion.  It  should 
be  borne  in  mind  that  the  vessels  of  the  fate  and  those  of  the  brain 
are  controlled  by  separate  mechanisms. 

Passive  hyperemias  result  from  mechanical  obstruction  to  the 
jugulars,  chiefly  from  new  growths,  emphysema,  and  loss  of  compensa- 
tion of  heart  disorders.  Pressure  of  the  head,  abnormal  wakefulness 
or  sleepiness,  dizziness,  apathy  or  mild  confusion,  with  anxiety,  are 
the  chief  signs,  combined  with  definite  signs  of  passive  congestion  of 
the  skin,  face,  and  mucous  membranes. 

Efficient  therapy  consists  in  ameliorating  the  causative  factors 
whether  iactive  or  passive.  The  hyperemias  are  purely  secondary. 
Acute  states  may  call  for  bleeding,  mustard  foot-baths,  active  catharsis. 

(6)  Cerebral  Arteriosclerosis. — This  is  a  widespread  disorder  occur- 
ring chiefly  after  forty  years  of  age  when  with  increasing  years  it  tends 


CEREBRAL  ARTERIOSCLEROSIS  469 

to  become  more  and  more  prominent.  The  arteriosclerotic  process 
differs  little  in  cerebral  vessels  from  those  located  in  other  organs  of 
the  body,  save  in  a  tendency  for  a  marked  production  of  miliary 
aneurisms.  These  are  largely  conservative  formations  and  are  not 
to  be  considered  as  productive  of  hemorrhage,  as  taught  by  Charcot 
and  Bouchard.^  Cerebral  arteriosclerosis  is  due  to  the  same  causes 
that  produce  arteriosclerosis  elsewhere  (heredity,  syphilis,  alcohol,  etc.), 
and  may  be  the  expression  of  a  general  disease,  or  may  be  sharply 
delimited  to  the  cerebral  vessels. 

Symptoms. — The  symptoms  of  early  arteriosclerosis  may  be  general 
or  localized.  The  chiefly  early  symptoms  are  sleeplessness,  restless- 
ness, headaches,  especially  if  there  are  dizzy  attacks,  and  renal 
changes,  neural  fatigue,  increased  emotionalism  and  irritability.  To 
this,  at  times  is  added  hebetude,  ready  forgetting,  especially  of  new 
impressions,  increased  blood-pressure.  These  signs  are  often  loosely 
spoken  of  as  neurasthenic.  Local  signs  may  be  added,  such  as  tem- 
porary lapses,  marked  somnolence,  tingling  or  numbness  or  other  signs 
of  focal  disease. 

The  chief  neurological  interests  are  focussed  upon  the  distribution 
of  the  focal  lesions  and  are  here  discussed  according  to  the  syndromes 
presented.    The  psychiatric  features  are  also  dwelt  upon  here  as  well 
as  in  the  special  groupings  under  the  senile  and  presenile  psychoses. 
These  chief  syndromes  are: 
I.  Disorders  due  to  disease  of  the  twig  and  terminal  branches. 

(a)  Cortical    branches:     irritative    complexes,    Alzheimer's 
disease. 

(h)  Medullary  branches:  lacunar  complex. 

(c)  Diffuse  types  (arteriosclerotic  dementias). 
II.  Disorders  of  chief  branches. 

(a)  Anterior  cerebral  syndromes,  crural  palsies. 

{h)  Middle  cerebral  syndromes. 

1.  Palsies:  hemorrhage,  embolism,  thrombosis,  palsies 

apoplexy. 

(1)  Monoplegias:  facial,  brachial. 

(2)  Combined  palsies. 

(3)  Complete  palsies:    capsular  and    supracap- 

sular. 

(4)  Thalamic  syndromes. 

2.  Aphasias. 

3.  Hemianopsias. 

(c)  Posterior  cerebral  syndromes. 

(d)  Inferior,  superior,  posterior,  cerebellar  syndromes. 

III.  Disorders  of  large  trunks  from  obliterating  lesions. 

1.  Basilar  syndromes. 

IV.  Senile  dementias,  not  necessarily  arteriosclerotic.^ 

1  See  Pick,  Berl.  kl.  Woch.,  February  21,  1910,  p.  325,  for  bibliography. 

2  Lambert,  States  Hospital  Bull.,  1908,  i,  475. 


470  DISEASES  OF  THE  BRAIN 

Clean-cut  examples  of  these  syndromes  are  rare.  The  patients 
present  mixed  conditions  save  in  the  few  instances  of  embolism  due 
to  the  closure  of  a  main  trunk.  For  this  reason  the  general  rather 
than  the  special  type  of  apoplectic  attack  will  be  described.  Special 
indications  as  to  the  localization  of  the  region  involved  will  be  noted 
later.  Pontine  and  midbrain  localizations  have  already  been  dis- 
cussed, as  have  also  disorders  of  the  cerebellum. 

Cerebral  Apoplexy. — ^Hemorrhage,  Thrombosis,  Embolism. — Men  are 
more  often  affected  (seven  to  five)  than  women,  and  four-fifths  of 
the  cases  occur  after  forty  years  of  age.  Arteriosclerosis,  as  indicated, 
plays  the  more  important  role  in  hemorrhage  and  in  thrombosis,  and 
the  smaller,  rather  than  the  basal,  vessels  are  responsible.  Cardiac 
hypertrophy  with  increased  blood-pressure  (180  to  225  mm.)  is  the 
chief  determining  factor,  and  is  closely  associated  with  the  arterio- 
sclerotic process.  Chronic  atrophic  kidney  disease  is  also  a  frequent 
concomitant  factor  (30  per  cent.).  Exciting  causes,  such  as  great 
physical  exertion,  lifting  heavy  weights,  coitus,  vomiting,  coughing, 
sneezing,  etc.,  were  formerly  given  a  prominent  place.  It  is  doubtful 
if  they  have  much  importance.  In  a  large  series  of  cases  studied  by 
Jones,  in  a  considerable  number  the  stroke  occurred  within  a  few 
minutes  after  getting  out  of  bed,  so  that  the  sudden  change  in  blood- 
pressure  on  awakening  and  getting  about  was  chiefly  responsible. 
Many  patients  develop  apoplexy  during  sleep,  although,  other  things 
being  equal,  sleep  is  a  protection. 

The  localization  of  the  hemorrhage  (thrombus)  varies  greatly. 
Right  and  left  sides  are  about  equally  involved.  Morgagni's  early 
dictum  concerning  the  greater  prevalence  of  right-sided  hemorrhages 
has  little  support  from  extensive  statistics.^ 

The  chief  syndromes  are  of  the  middle  cerebral  type.  The  branches 
of  the  lenticulostriate  arteries  supplying  the  internal  capsule,  caudate 
nucleus,  lenticular  nucleus,  and  optic  thalamus  are  most  frequently 
involved.  Thus  the  most  frequent  syndromes  are  the  combined  palsies, 
arm  and  leg;  arm,  leg,  and  face;  arm,  leg,  face,  with  sensory  symptoms 
and  aphasic  complexes. 

Cortical  hemorrhages  are  probably  much  more  frequent  than  is 
usually  supposed  since  many  occur  without  the  death  of  the  patient. 
These  result  in  more  limited  syndromes,  such  as  apraxias,  arm  mono- 
plegias (anterior  cerebral  syndrome). 

Monoplegias  of  the  leg,  isolated  aphasias,  lower  quadrant  hemianop- 
sias, and  the  thalamic  syndrome  belong  more  especially  to  the  middle 
cerebral  syndrome,  while  mind-blindness,  and  homonymous  hemianop- 
sias are  the  chief  features  of  the  isolated  posterior  cerebral  syndromes.    . 

Midbrain,  pontine,  medullary,  and  cerebellar  hemorrhages  are 
comparatively  infrequent,  and  have  a  special  syndromy  described 
elsewhere. 

1  See  995  cases  collected  by  Jones,  Brain,  1905. 


APOPLEXY 


471 


The  Apoplectic  Attack. — Nausea  and  vomiting  are  the  most  frequent 
precursors  of  the  apoplectic  attack.    In  thrombosis  or  emboKc  occlud- 


FiG.  231. — Traumatic  supradural  hemorrhage.     (Larkin.') 


Fig.  232. — Cerebral  hemorrhage  within  the  ventricle. 


472  DISEASES  OF   THE  BRAIN 

ing  lesions,  twitchings  or  even  convulsions  are  more  frequent  than  in 
hemorrhagic  cases.  In  hemorrhagic  cases  with  convulsions  the  bleeding 
is  more  liable  to  have  extended  to  the  ventricles,  and  can  freciuently 
be  demonstrated  by  lumbar  puncture.    This  is  not  invariable,  however. 

Loss  of  consciousness  is  usual,  especially  in  hemorrhagic  cases,  less 
often  present  in  thrombosis  or  embolism,  especially  in  the  beginning. 
In  Jones'  series,  47.7  per  cent,  of  cases  of  embolism  showed  loss  of 
consciousness,  impairment  in  60  per  cent.  Thromboses  show  a  similar 
percentage,  while  hemorrhage  is  accompanied  by  loss  of  conscious- 
ness in  75  per  cent,  of  the  cases.  These  figures  are  of  minor  value  in 
determining  a  differential  diagnosis. 

The  attack  is  usually  abrupt.  Dizziness,  heaviness,  anxiety,  head- 
ache, paresthesise  may  be  described  by  those  who  do  not  at  first  become 
suddenly  unconscious,  and  yet  in  whom  a  gradually  developing  state 


Fig.  233. — Cystic  hemorrhage  of  hypophysis.      (Larkin.) 

of  unconsciousness  occurs,  with  paraphasia  and  gradual  weakness  of 
one  side  of  the  body.  Many  patients  are  able  to  describe  the  begin- 
ning symptoms  with  accuracy  after  recovery  from  even  a  profound 
coma  lasting  a  week  or  more. 

Coma  developing  is  accompanied  by  stertorous  breathing,  by  slow, 
full,  regular  pulse.  The  patient  is  pale,  or  the  face  may  be  congested, 
the  extremities  are  cold.  The  limbs  are  completely  relaxed.  Minute 
variations  in  the  excursion  of  the  two  sides  of  the  chest,  irregularity 
of  the  usually  dilated  and  light-inactive  pupils,  minor  signs  of  irrita- 
bility (Marie-Foix)  may  enable  one  to  locate  the  side  of  the  hemorrhage 
even  in  this  comatose  state.  Other  signs  are  loss  of  corneal  reflexes, 
no  reaction  to  painful  stimuli,  loss  of  reflexes  in  general,  occasionally 
involuntary  urination  and  defecation.    Retention  is  more  apt  to  occur. 

In  severe  states  of  coma  with  marked  rise  in  temperature,  103°  F.  to 
105°  F.,  with  twitching  or  convulsive  jerks,  very  slow  heart  action,  later 


APOPLEXY  ^  473 

developing  cardiac  and  pulmonary  symptoms,  the  hemorrhage  is 
probably  very  extensive,  involves  the  ventricles,  and  death  ensues 
in  a  few  hours  or  a  few  days.  Thalamic  cases  are  frequently  attended 
with  marked  coma,  which  may  be  very  protracted,  three  to  four  weeks, 
without  definite  hemiplegic  signs. 

Jacksonian  convulsive  attacks  usually  indicate  meningeal  bleeding. 
Some  patients  show  a  tendency  to  clouding  of  consciousness  which 
comes  and  goes. 

Recovery  from  the  immediate  attack  may  take  place  in  a  few  hours 
or  after  weeks  or  more.  The  residuals  found  will  vary  according  to 
the  location  and  extent  of  the  lesion.  It  is  in  this  stage  that  a  diagnosis 
of  the  localization  and  extent  of  the  lesion  can  be  made. 

In  emboli,  thrombi,  and  hemorrhage  of  the  anterior  cerebral  arteries 
the  lesion  may  be  limited  or  very  extensive,  Apraxia  may  be  an  iso- 
lated symptom,  or  there  may  be  combined  apraxia  and  crural 
monoplegia. 

Mild  or  severe  and  acute  confusions  with  variously  colored  mental 
defect  states  are  frequently  the  result  of  minute  or  more  extensive 
changes  in  the  vascular  supply  of  the  frontal  lobes,  also  supplied  by 
the  anterior  cerebrals.  These  patients  not  infrequently  present  lacunar 
symptoms  when  the  medullary  vessels  are  involved.^  The  clinical 
picture  is  protean,  depending  on  the  severity  of  the  hemorrhage,  or 
softening  (from  embolus  or  thrombus)  and  the  distribution  of  these  focal 
softenings.  The  course  is  more  halting;  epileptiform  or  apoplectiform 
attacks,  with  acute  confusions  occur,  from  which  the  patient  is  apt  to 
make  a  clinical,  not  anatomical,  recovery.  The  patient  is  usually 
aged  forty,  fifty,  or  sixty  years.  There  is  gradually  increasing  sense 
of  incompetency.  Vertiginous  attacks  occur,  with  headaches,  often 
migrainous  in  character.  Speech  and  thought  associations  are  inter- 
fered with  and  minor  monoplegias  develop.  In  lacunar  syndromes, 
apraxias,  and  hallucinoses  result  from  arteriocerebral  lesions  chiefly; 
aphasias,  agraphias,  hemiplegias,  hemianesthesias  from  middle  cere- 
bral lesions.  General  progression  may  result  with  marked  mental 
enfeeblement — arteriosclerotic  dementias — or  a  sudden  new  and  more 
extensive  lesion  may  cause  death. 

Irritative  complexes  from  cortical  vascular  disease  develop  not 
infrequently,  even  in  3'ounger  individuals  following  various  infections 
(thrombi,  emboli  of  cortical  vessels),  alcoholism,  syphilis,  lead.  Here 
transitory  mild  shock  may  usher  in  the  disorder  with  twitching,  jerky, 
choreiform  accompaniments,  or  these  motor  disturbances  may  later 
develop  as  residuals,  with  or  without  epileptiform  attacks.  Pares- 
thesise,  astereognosis,  weakness  of  an  arm  or  leg,  or  mjld  paresis  of  the 
facial  musculature  may  develop.  True  epileptic  residuals  may  be 
permanent  symptoms  of  certain  of  these  cortical  arteriosclerotic  focal 
lesions. 

'  Lambert,  States  Hosp.  Bull.,  1909,  ii,  459. 


474  DISEASES  OF   THE  BRAIN 

The  middle  cerebral  syndromes  are  the  most  frequent.  Here  hemi- 
plegia is  the  most  striking  residual  symptom.  In  total  hemiplegia, 
usually  capsular,  supracapsular,  the  face,  arm,  and  leg  of  the  same  side 
are  involved.  The  upper  branches  of  the  facial,  wrinkling  of  forehead, 
closure  of  eye  are  not  usually  involved.  Nor  are  the  eye  movements 
involved  unless  the  lesion  is  located  lower  down,  crura  or  in  the  mid- 
brain, when  another  syndrome  is  present.^  The  face  is  drawn  to  the 
healthy  side,  the  tongue  projects  to  the  paralyzed  side  on  protrusion. 
Dysphagia,  from  hemiparesis  of  the  palate  develops.  The  soft 
palate  hangs  lower  on  the  paralyzed  side.  The  neck  may  be  involved, 
but  is  less  apt  to  be,  whereas  the  paralysis  of  the  arm  and  leg  are 
characteristic.     (See  Plate  VIII.) 

The  arm  is  flaccid,  flabby,  apt  to  be  edejnatous,  blue,  cold,  and  boggy. 
The  reflexes  which  during  coma  are  lost,  gradually  return  and  shortly 
become  exaggerated.  Chaddock's  wrist  phenomenon  develops.  There 
is  little  or  no  atrophy,  and  the  electrical  reactions  are  normal.  Later 
contractions  may  develop,  or  may  be  early  if  the  lesion  involves  only 
a  portion  of  the  arm  fibers  or  is  apt  to  encroach  on  the  thalamus. 
The  abdominal  reflexes  are  diminished  or  lost  on  the  paralyzed  side. 

The  leg  is  flaccid,  is  thrown  about  the  hip  like  a  flail,  may  be  edema- 
tous and  flabby,  and  also  shows  later  the  signs  of  pyramidal  tract, 
upper  neuron  disorder,  i.  e.,  increased  knee-jerks,  ankle-clonus,  Babinski 
and  Chaddock  reflexes,  with  increased  muscle  tone  and  normal  electrical 
responses. 

The  grade  of  the  paralysis  varies  greatly.  With  some  it  is  a  transi- 
tory weakness  which  passes  in  a  few  weeks  or  a  few  months,  in  others 
it  is  a  complete  permanent  and  marked  paralysis,  with  later  develop- 
ing contractures  and  total  unilateral  disability.  Any  intermediary 
grade  may  be  expected.  In  the  milder  forms  of  hemiplegia  the  little 
signs — see  Examination  Methods — are  of  great  value  in  clearing  up 
the  situation,  since  some  mild  hemiplegias  may  be  confused  with 
psychogenic  palsies,  especially  in  thalamic  cases. 

Hemianesthesia  may  accompany  the  hemiplegia,  or  it  may  be  an 
isolated  symptom.  This  argues  for  the  implication  of  a  portion  of 
the  thalamic  fibers.  The  patients  complain  of  numbness,  coldness, 
and,  depending  on  the  location  of  the  lesion,  may  lose  their  stereognos- 
tic  sense,  may  not  be  able  to  distinguish  heat,  nor  cold,  or  may  have 
subjective  sensations,  haptic  hallucinations  of  the  limb  areas. 

Sensory  disturbances  from  cerebral  lesions  are  extremely  diverse 
and  important  from  the  diagnostic  point  of  view.  They  are  best 
discussed  with  a  consideration  of  the  thalamic  syndrome. 

The  Thalamic.  Syndrome.^ — If  the  thalamus  alone  is  involved  or 
with  partial  implication  of  related  extrapyramidal  tracts,  a  character- 
istic neurological  complex  develops,  termed  by  Dejerine  and  his  pupil 


1  Millard-Gubler;  Benedict,  etc.,  q.  v. 

2  Jelliffe,  The  Thalamic  Syndrome,  Medical  Record,  February  1,  1910,  for  references. 


THALAMIC  SYNDROME 


475 


Roussy  the  thalamic  syndrome.     It  is  one  of  the  middle  cerebral 
artery  syndromes. 

The  chief  features  show  usually  after  an  apoplectic  attack  with 
the  ordinary  signs  of  a  severe  hemorrhage,  thrombosis,  or  embolism. 
In  certain  syphilitic  cases  the  attack  may  be  comparatively  slight, 
or  the  syndrome  may  develop  with  no  signs  of  an  attack. 


F.  cortico- 
thalam. 


Dorsal  column 
sensory  paths 


Crossed  secondary 
sensory  paths 


Fig.  234. — To  show  the  position  and  relations  of  the  optic  thalamus  in  the  central 
sensory  path.  Two  distinct  paths  exist  in  the  spinal  cord;  a  crossed  secondary  path  in 
the  ventrolateral  column  which  conveys  impressions  of  pain,  temperature,  and  touch, 
and  a  second  uncrossed  path  in  the  dorsal  column  which  also  carries  touch,  and  in  which 
run  impulses  that  underlie  the  sense  of  position,  the  appreciation  of  movement,  the  dis- 
crimination of  two  points,  and  the  recognition  of  vibration,  size,  shape,  form,  weight, 
and  consistence.  This  second  path  decussates  in  the  lower  part  of  the  medulla  oblongata, 
but  runs  separate  from  the  first  path,  at  least  as  high  as  the  pons.  All  these  secondary 
sensory  fibers,  now  crossed,  terminate  in  the  ventrolateral  region  of  the  optic  thalamus. 
The  impressions  they  carry  are  regrouped  here  and,  through  intercalated  neurons,  are 
distributed  along  two  distinct  paths;  the  one  carries  impressions  to  the  cerebral  cortex, 
the  other  toward  the  more  mesial  parts  of  the  optic  thalamus.  The  corticothalamic 
fibers,  which  terminate  in  the  lateral  nucleus  of  the  optic  thalamus,  are  also  shown. 
(Head  and  Holmes.)      (Compare  Plate  XI.) 


Practically  the  entire  mass  of  sensory  fibers  carrying  impulses  of 
all  kinds — the  tests  for  most  of  which  have  already  been  outlined — 
have  synaptic  junctions  within  the  optic  thalamus.  No  discussion  is 
here  given  of  the  numerous  fibers  coming  from  the  chemical  receptors 
of  the  respiratory,  gastro-intestinal,  or  genito-urinary  tract,  nor  those 
from  the  organs  of  internal  secretion,  nor  even  of  the  sympathetic — 


476  DISEASES  OF   THE  BRAIN 

all  of  these  make  up  an  enormous  terra  incognita  in  the  thalamus  or 
related  structures. 

The  chief  features  of  the  thalamic  syndrome  are: 

LA  persistent  loss  of  superficial  sensation  of  one-half  of  the  body 
and  face.  This  loss  to  touch,  pain,  and  to  temperature  is  more  or 
less  definite,  subject  to  considerable  variation  and  to  partial  recovery, 
but  the  loss  of  deep  sensibility,  deep  pressure,  postural  sense,  etc., 
is  much  more  pronounced,  and  is  more  apt  to  persist.  This  latter 
is  usually  more  marked  distally  and  in  many  instances  diminishes 
proximally. 

2.  There  is  slight  hemiataxia  and  more  or  less  complete  astereognosis. 

3.  There  are,  in  the  complete  syndrome,  acute  pains  on  the  affected 
side,  which  are  very  persistent,  coming  on  in  paroxysms.  They  are 
frequently  extremely  severe  and  rarely  respond  to  the  ordinary  anal- 
gesics. These  pains  may  involve  a  single  member,  may  be  limited 
to  the  side  of  the  face,  simulating  a  trigeminal  neuralgia,  or  they  may 
involve  one  whole  side  of  the  bod3\ 

4.  There  is  usually  a  more  or  less  distinct,  though  slight,  hemiplegia 
which,  in  the  unmixed  syndromes,  rapidly  clears  up.  Contractures 
rarely  develop  in  the  pure  syndrome.  In  the  mixed  syndrome — with 
extension  of  the  lesion  to  the  internal  capsule — contractures  may  be 
present. 

5.  Choreic,  athetoid,  or  paralysis  agitans-like  movements  may  be 
present  on  the  affected  side. 

These  are  the  chief  symptoms  of  optic  thalamus  disease,  but  in 
addition  to  these,  Head  and  Holmes  have  pointed  out  an  extremely 
suggestive  series  of  affective  reactions  which  are  due  to  lesions  which 
cut  the  optic  thalamus  from  its  cortical  connections.  They  have 
opened  up  an  attack  upon  the  analysis  of  the  sensory  content  of 
emotional  reactions.  They  show  that  in  this  type  of  lesion  there  is 
a  tendency  to  react  excessively  to  unpleasant  stimuli.  The  prick  of 
a  pin,  painful  pressure,  excessive  heat  or  cold,  all  produce  more  dis- 
tress than  on  the  normal  half  of  the  body.  Thus,  in  one  of  Head  and 
Holmes'  patients,  if  a  pin  was  dragged  lightly  across  the  face,  or  trunk, 
from  the  sound  to  the  affected  side,  there  was  felt  an  excessive  discom- 
fort as  it  passed  the  middle  line.  She  not  only  complained  that  it 
hurt  her,  but  the  face  was  contorted  with  pain,  and  all  this  notwith- 
standing the  fact  that  she  was  less  able  to  distinguish  head  from  point, 
yet  the  prick  hurt  her  more.  This  very  anomalous  state  of  affairs 
is  a  purely  thalamic  reaction. 

This  excessive  reactivity  is  subject  not  only  to  pin-prick,  but  also 
to  deep  pressure,  to  extremes  of  heat  and  cold,  to  visceral  stimulation, 
to  scraping,  roughness,  vibration,  tickling,  to  pleasurable  stimuli, 
and  to  ideational  emotional  states.  Not  all  patients  show  all  of  these 
reactions,  but  in  practically  90  per  cent,  of  the  thalamic  cases  examined 
by  Head  and  Holmes,  excessive  affective  response  to  one  or  more 
measured  stimuli  were  found.    For  heat  and  cold,  and  other  forms  of 


THALAMIC  SYNDROME  477 

sensibility  as  well  as  for  pain,  the  excessive  response  may  be  present, 
and  yet  the  patients  are  unable  to  detect — ?'.  e.,  are  anesthetic  to — the 
stimulus  itself.  So  far  as  the  ideational  affective  reaction  is  concerned, 
these  patients  express  themselves  as  follows:  On  hearing  affecting 
music,  "a  horrid  feeling  came  on  in  the  affected  side,  and  the  leg 
screwed  up  and  started  to  shake."  The  singing  of  a  comic  song  left 
one  patient  absolutely  cold,  but  a  tragic  song  produced  a  very  distinct 
unpleasant  effect.  One  patient  said,  "my  right  hand  seems  to  crave 
sympathy,  my  right  side  seems  more  artistic."  In  practically  all  of 
the  cases  the  increased  affective  reaction  was  accompanied  by  actual 
sensory  loss. 

A  more  detailed  study  of  the  loss  of  sensibility  in  thalamic  disorders 
made  by  Head  and  Holmes  revealed  the  following:  No  sensory  func- 
tions are  so  frequently  affected  as  the  appreciation  of  posture  and  the 
recognition  of  passive  movement.  The  amount  of  this  loss  varies 
greatly  from  a  scarcely  measurable  defect  to  complete  want  of  recog- 
nition of  the  posture  of  the  limbs  of  the  abnormal  half  of  the  body. 

Tactile  sensibility  is  frequently  diminished,  but,  excepting  in  a  few 
cases,  where  all  appreciation  of  contact  was  destroyed,  a  threshold 
could  be  obtained.  It  was  always  possible  to  show  that  increasing 
the  strength  of  the  stimulus  improved  the  proportion  of  right  answers 
unless  the  observations  were  confused  by  the  disagreeable  tingling  or 
other  accessory  sensations. 

Localization  of  the  spot  touched  was  defective  in  half  the  cases  where 
sensation  was  sufficiently  preserved  to  carry  out  accurate  tests.  This 
inability  to  recognize  the  site  of  simulation  w^as  equally  great,  whether 
the  patient  was  pricked  or  touched.  In  cases  where  localization  was 
gravely  affected,  the  disagreeable  sensation,  so  easily  evoked,  tended 
to  spread  widely  on  the  abnormal  half  of  the  body.  A  prick  on  the 
hand  may  cause  an  extremely  painful  sensation  in  the  cheek  or  side, 
and  sometimes  the  patient  simply  recognized  the  stimulus  as  a  change 
within  himself,  and  did  not  refer  the  discomfort  from  which  he  suffered 
to  the  action  of  any  external  agent. 

Sensibility  to  heat  and  cold  may  show  all  degrees  of  change  from 
total  loss  to  a  slight  increase  of  the  neutral  zone.  Heat  and  cold  are 
not  dissociated;  and  if  one  form  of  sensation  is  lost,  the  other  will 
be  gravely  disturbed.  The  apparent  exceptions  arise  from  a  misin- 
terpretation of  the  sensation  evoked  by  high  or  low  temperatures  on 
the  affected  half  of  the  body. 

Not  infrequently  the  compass  test  cannot  be  carried  out  because 
of  the  gross  loss  of  sensation  and  inability  to  recognize  contact;  but 
whenever  this  method  can  be  applied  a  threshold  can  be  worked  out, 
and  widening  the  distance  between  the  points  increases  the  accuracy 
of  the  answers. 

The  power  of  estimating  the  relation  between  two  weights  is  fre- 
quently disturbed  on  the  abnormal  half  of  the  body.  If  the  apprecia- 
tion of  posture  and  movement  is  affected,  the  patient  can  no  longer 


478  DISEASES  OF   THE  BRAIN 

recognize  the  identity  or  the  difference  of  two  weights  placed  in  the 
unsupported  hands.  But  so  long  as  tactile  sensibility  is  not  dimin- 
ished, he  can  still  estimate  the  relation  between  weights  applied  one 
after  the  other  to  the  same  spot,  and  can  recognize  the  increase  or 
diminution  in  weight  of  an  object  already  resting  on  the  hand. 

The  appreciation  of  relative  size  is  often  disturbed  in  these  cases. 
With  care  it  is  usually  easy  to  demonstrate  a  difference  in  the  threshold. 
Shape  and  form  in  three  dimensions  are  frequently  not  recognizable 
on  the  affected  hand.  But,  if  tactile  sensibility  is  not  grossly  affected, 
the  patient  usually  retains  an  idea  that  the  object  possesses  a  form, 
and  may  give  a  considerable  percentage  of  right  answers. 

Vibration  of  the  tuning-fork  in  almost  all  thalamic  cases  is  felt, 
but  the  length  of  time  during  which  it  is  appreciated  is  usually 
shorter,  and  sometimes  the  rate  of  vibration  is  thought  to  be  slower 
on  the  affected  half  of  the  body. 

Roughness,  as  tested  with  Graham  Brown's  esthesiometer  is  usu- 
ally recognized,  except  in  those  cases  in  which  the  loss  of  all  forms  of 
sensation  is  unusually  severe. 

Partial  syndromes  are  more  frequent  than  the  classical  syndrome 
described.  To  summarize:  the  main  symptoms  of  the  complete 
syndrome  are: 

1.  Persistent  hemianesthesia,  more  or  less  marked  for  superficial 
sensibility,  tactual  pain,  temperature,  most  marked  for  deep  sensi- 
bility, and  hence  loss  of  postural  sense  and  astereognosis. 

2.  Mild  hemiplegia,  usually  regressive. 

3.  Mild  hemiataxia,  with  choreo-athetoid  movements  either  spon- 
taneous or  on  an  attempted  movement.     Adiadokokinesia  at  times. 

4.  Paroxysmal,  shooting,  neuralgic  pains,  often  very  persistent  and 
severe  and  not  helped  by  the  usual  analgesics. 

5.  Over-response  to  affective  reactions,  particularly  on  affected  side, 
with  emotional  hyperactivity  to  varied  sensory  impressions. 

The  analysis  of  sensory  stimuli  in  cerebral  and  thalamic  disorders 
is  of  the  greatest  importance  in  cerebral  localization,  hence  an  extended 
presentation  of  contemporary  work  is  desirable,  particularly  that  of 
Head  and  Holmes. 

Sensory  Changes  in  Cortical  Lesions,  Supra-thalamic  Pathways. — 
In  the  chapters  on  Peripheral  Nerves  the  chief  sensory  syndromes  of 
the  first  sensory  neurons  were  discussed.  Those  of  the  cord  are  pre- 
sented elsewhere.  Redistributions  take  place  in  the  medulla  and  mid- 
brain. Those  disturbances  due  to  lesions  within  the  thalamus  have 
just  been  presented.  Finally  the  thalamocortical  pathways  make  a 
fifth  regrouping  of  sensory  phenomena  in  the  entire  course  of  the 
sensory  neuron.     (See  Plates  X  and  XI.) 

The  analysis  of  these  phenomena  introduces  complex  factors,  and 
it  is  necessary  to  abandon  all  generalizations,  even,  for  instance,  light 
touch,  cutaneous  sensation.  The  results  of  the  tests  must  be  stated 
in  terms  of  the  tests  employed.    (See  Sensory  Examination  Methods.) 


CORTICAL  SENSORY  DISTURBANCES  479 

Using  graduated  tactile  stimuli,  such  as  von  Frey's  hairs  and  the 
pressure  esthesiometer,  the  chief  results  obtained  by  Head  and  Holmes 
and  others  have  been  that  a  cortical  lesion  may  reduce  the  accuracy 
of  response  from  the  affected  part  to  graduated  tactile  stimuli.  The 
form  assumed  by  this  defective  sensibility  differs  from  that  produced 
by  lesions  at  other  levels  of  the  nervous  system.  The  affected  part 
may  respond  to  the  same  graduated  hair  as  the  normal  hand;  but 
this  response  is  irregular  and  uncertain.  Increasing  the  stimulus  may 
lead  to  no  corresponding  improvement,  and  even  the  strongest  tactile 
hair  may  occasionally  evoke  less  certain  answers  than  a  hair  of  much 
smaller  bending  strain.  Moreover,  a  touch  with  the  unweighted 
esthesiometer  may  be  as  effective  at  one  moment  as  the  same  instru- 
ment weighted  with  30  grams  at  another.  In  such  cases  no  tactile 
threshold  can  be  definitely  obtained.  This  irregularity  of  response 
is  associated  with  persistence  of  the  tactile  sensation  and  a  tendency  to 
hallucinations  of  touch.  Where  the  sensory  defect  is  not  sufficiently 
gross  to  abolish  the  threshold,  persistence,  irregularity  of  response 
and  a  tendency  to  hallucinate  may  still  disturb  the  records. 

In  all  cases  where  tactile  sensibility  is  affected,  whether  a  threshold 
can  be  obtained  or  not,  fatigue  is  induced  with  unusual  facility. 
Although  the  patient  may  cease  to  respond  to  tactile  stimuli  over  the 
affected  part  in  consequence  of  fatigue,  his  answers  may  remain  as 
good  as  before  from  the  normal  parts.  The  fatigue  is  local  and  not 
general. 

With  stationery  cortical  lesions,  uncomplicated  by  states  of  shock 
or  by  "diaschisis,'^  sensibility  to  touch  with  cotton-wool  is  never 
lost  over  hair-clad  parts.  Over  hairless  parts,  stimulation  with  cotton- 
wool may  produce  a  sensation  which  seems  ''less  plain"  to  the  patient, 
and  his  answers  may  show  the  same  inconstancy  so  evident  when 
he  is  tested  with  graduated  tactile  stimuli. 

A  pure  cortical  lesion  leads  to  no  change  in  the  threshold  to 
measurable  painful  or  uncomfortable  stimuli.  Nor  does  the  patient 
express  greater  dislike  to  these  stimuli  on  one  side  than  on  the  other. 
A  prick  may  be  said  to  be  "plainer"  or  "sharper"  on  the  normal 
than  on  the  affected  side;  but  this  is  due  to  a  defective  appreciation 
of  the  pointed  nature  of  the  stimulus  and  bears  no  direct  relation  to 
the  painfulness  of  the  sensation  evoked. 

Temperature  tests  show  that  (a)  the  neutral  zone,  within  the  stimu- 
lus, was  said  to  be  neither  hot  nor  cold,  was  considerably  enlarged  in 
comparison  with  that  observed  on  similar  normal  parts  of  the  same 
patient,  (h)  The  patient  complained  that,  although  he  recognized 
correctly  the  nature  of  the  stimulus,  it  seemed  "less  plain"  than  over 
normal  parts.  His  answers  were  less  constant,  and  less  certain;  a 
temperature  recognized  without  difficulty  at  one  time  seemed  doubt- 
ful at  another,  (c)  The  power  of  discriminating  the  relative  coolness 
of  two  stimuli  or  the  relative  warmth  of  two  hot  tubes  may  be  dimin- 
ished.   Thus  20°  C.  may  be  said  to  be  the  same  as  ice,  although  both 


480  DISEASES  OF   THE  BRAIN 

are  uniformly  called  cold,  and  40°  C,  may  seem  as  warm  as  or  even 
warmer  than  48°  C.  The  faculty  of  appreciating  the  relation  to  one 
another  of  two  temperatures  on  the  same  side  of  the  scale  is  disturbed. 

Tests  for  ijosture  and  for  -passive  movements  show  that  («)  cortical 
lesions  most  frequently  disturb  the  recognition  of  posture  and  of  pas- 
sive movements.  Whenever  sensation  is  in  any  way  affected  in  con- 
sequence of  a  cortical  lesion  these  two  functions  suffer.  (6)  In  all 
their  cases  the  disturbance  in  the  faculty  of  recognizing  posture  and 
passive  movements  was  greater  toward  the  peripheral  parts  of  the 
affected  limb,  (c)  When  a  patient  with  unilateral  disturbance  of  these 
faculties  attempts  to  point  to  some  part  of  his  body,  defective  knowl- 
edge of  its  position  causes  greater  error  than  want  of  recognition  of 
posture  and  movement  in  the  hand  with  which  he  points,  {d)  When 
testing  the  patient's  power  of  appreciating  passive  movement,  the 
answers  are  frequently  uncertain  and  hallucinations  of  movement 
may  occur.  And  yet  the  patient  may  be  remarkably  consistent  and 
accurate  when  normal  parts  are  tested. 

Localization  tests  show  that  (a)  the  power  of  localizing  the  stimulated 
spot  is  not  infrequently  preserved,  although  sensation  may  be  other- 
wise disturbed  as  a  consequence  of  cortical  lesions,  {h)  This  faculty 
is  independent  of  the  power  of  recognizing  the  position  of  the  affected 
limb;  appreciation  of  posture  may  be  lost,  although  localization  is 
not  in  any  way  diminished,  (c)  If  the  power  of  localization  is  lost,  the 
patient  will  be  unable  to  recognize  not  only  the  position  of  a  spot 
touched  but  also  the  position  of  a  prick,  {d)  When  localization  is 
defective  in  consequence  of  cerebral  lesions,  the  patient  does  not 
habitually  localize  in  any  particular  direction,  but  ceases  to  be  certain 
where  he  has  been  touched  or  pricked. 

The  compass  test  shows  that  (a)  a  cortical  lesion  may  destroy  the 
power  of  discriminating  two  compass  points,  both  when  applied  simul- 
taneously and  collectively.  If  this  is  the  case,  no  threshold  can  be 
obtained  for  either  form  of  the  test;  increasing  the  distance  between 
the  points  does  not  constantly  improve  the  accuracy  of  the  answers. 
(6)  This  disturbance  is  not  caused  by  changes  in  tactile  appreciation; 
for  it  can  be  demonstrated  equally  well  with  two  painful  as  with  two 
tactile  stimuli,  (c)  The  condition  of  tactile  sensibility  and  the  accu- 
racy of  the  simultaneous  compass  test  are  closely  associated;  a  dis- 
turbance of  the  tactile  threshold  is  usually  accompanied  by  a  raised 
threshold  for  the  appreciation  of  two  points  applied  simultaneously. 
{d)  Should  the  power  be  preserved  of  recognizing  two  points  when  the 
compasses  are  applied  consecutively,  localization  will  be  found  to  be 
intact.  The  patient's  appreciation  of  the  two  points  when  they  are 
separated  by  an  interval  of  time  is  due  to  the  recognition  of  the 
separate  locality  of  the  two  spots  touched. 

Appreciation  of  weights  shows  that  (a)  the  power  of  estimating  the 
relation  of  two  objects  of  the  same  size  and  shape  is  readily  disturbed 
by  cortical  lesions.     (6)  Though  the  patient  may  retain  sensations  of 


CORTICAL  SENSORY  DISTURBANCES  481 

contact  when  the  weight  is  placed  in  his  hand,  all  power  of  recognizing 
the  relative  heaviness  of  the  object  has  disappeared,  (c)  This  faculty 
is  equally'  disturbed  in  most  cases  whether  the  weights  are  placed  on 
the  supported  or  the  unsupported  hand. 

From  these  and  related  studies,  Head  and  Holmes  maintain  that 
sensory  impulses  pass  from  the  thalamus  to  the  cortex  in  five  groups : 

1.  Those  concerned  with  the  recognition  of  posture  and  passive 
movement.  If  these  impulses  are  affected  the  power  of  discriminating 
weights  on  the  unsupported  hand  may  be  also  diminished. 

2.  Certain  tactile  elements;  integrity  of  this  group  is  necessary 
for  the  discrimination  of  weights  placed  on  the  fully  supported  hand. 

3.  Those  impulses  which  underlie  the  appreciation  of  two  points 
applied  simultaneously  (the  compass  test) ;  on  this  group  also  depends 
the  recognition  of  size  and  shape. 

4.  Those  which  underlie  the  power  of  localizing  the  situation  of  a 
stimulated  spot.  Recognition  of  the  double  nature  of  two  points 
applied  consecutively  also  depends  on  this  group  of  impulses. 

5.  All  thermal  impulses  are  grouped  together  to  underlie  a  scale  of 
sensations  with  heat  at  the  one  end  and  cold  at  the  other.  At  the 
level  with  w^hich  we  are  now  dealing  these  impulses  have  already 
excited  the  affective  center  and  are  passing  away  to  the  cortex. 

The  functional  integrity  of  the  cortex  enables  attention  to  be 
concentrated  upon  those  changes  which  are  produced  by  the  arrival 
of  afferent  impulses  (Head  and  Holmes).  When  this  is  disturbed, 
some  impulses  evoke  a  sensation,  but  others,  from  lack  of  attention, 
do  not  affect  consciousness.  Attention  no  longer  moves  freely  over 
the  sensory  field  to  be  focussed  successively  on  fresh  groups  of  sensory 
impressions.  Sensations,  once  evoked,  are  not  cut  short  by  the  moving 
away  of  the  focus  of  attention  as  wdien  cortical  activity  is  perfect. 
Hence  arise  persistent  sensations  and  hallucinations  which  are  so 
prominent  a  feature  of  lesions  of  the  cortex. 

The  cerebral  cortex  is  the  organ  by  which  we  are  able  to  focus 
attention  upon  the  changes  evoked  by  sensory  impulses.  A  pure 
cortical  lesion,  which  is  not  advancing  or  causing  periodic  discharges, 
will  change  the  sensibility  of  the  affected  parts  in  such  a  way  that 
the  patient's  answers  appear  to  be  untrustworthy.  Such  diminished 
power  makes  the  estimation  of  a  threshold  in  many  cases  impossible. 
Uncertainty  of  response  destroys  all  power  of  comparing  one  set  of 
impressions  with  another  and  so  prevents  discrimination. 

But  in  addition  to  its  function  as  an  organ  of  local  attention  the 
sensory  cortex  is  also  the  storehouse  of  past  impressions.  These  may 
rise  into  consciousness  as  images,  but  more  often,  as  in  the  case  of 
spacial  impressions,  remain  outside  central  consciousness.  Here 
they  form  organized  models  of  ourselves  which  may  be  termed  ''  sche- 
mata." Such  schemata  modify  the  impressions  produced  by  incoming 
sensory  impulses  in  such  a  way  that  the  final  sensations  of  position, 
or  of  locality,  rise  into  consciousness  charged  wdth  a  relation  to  some- 
31 


482  DISEASES  OF   THE  BRAIN 

thing  that  has  happened  before.  Destruction  of  such  "schemata" 
by  a  lesion  of  the  cortex  renders  impossible  all  recognition  of  posture 
or  of  the  locality  of  a  stimulated  spot  on  the  affected  part  of  the  body. 

In  daily  life  all  stimuli  excite  more  or  less  both  thalamic  and  cor- 
tical centers,  for  most  unselected  sensations  contain  both  affective 
and  discriminative  elements.  But  among  the  tests  employed  in  sen- 
sory analysis,  some  appeal  almost  entirely  to  the  one  or  the  other 
center.  The  test  for  recognition  of  posture,  as  carried  out  by  Head 
and  Holmes,  is  purely  discriminative;  while  the  pain  produced  by 
squeezing  the  testicle,  or  to  a  less  degree  by  the  pressure  algometer, 
appeals  almost  exclusively  to  the  more  affective  center. 

Sensory  impulses  arriving  at  the  optic  thalamus  are  regrouped 
in  such  a  way  that  they  can  act  upon  both  its  essential  center  and  the 
sensory  cortex.  The  essential  organ  of  the  thalamus  is  excited  to 
affective  activity  by  certain  impulses,  and  refuses  to  react  to  those 
which  underlie  the  purely  discriminative  aspects  of  sensation.  These 
pass  on  to  influence  the  cortical  centers  where  they  are  readily 
accepted.  In  a  similar  way,  the  primary  centers  of  the  cortex  cannot 
receive  those  components  which  underlie  feeling  tone;  in  this  direc- 
tion they  are  completely  blocked. 

It  has  long  been  recognized  that  sensations  are  endowed  with  feel- 
ing tone  to  different  degrees.  In  those  which  underlie  postural 
appreciation  this  quality  is  entirely  absent,  while  visceral  sensations 
are,  in  some  instances,  little  more  than  a  change  in  a  general  feeling 
tone;  the  former  set  of  impulses  appeals  almost  exclusively  to  the 
cortical  center,  the  latter  to  that  of  the  optic  thalamus.  All  thermal 
stimuli,  however,  make  a  double  appeal.  Every  sensation  of  heat  or 
cold  is  either  comfortable  or  uncomfortable;  the  only  entirely  indif- 
erent  temperature  is  one  that  is  neither  hot  nor  cold. 

In  the  same  way,  some  unselected  tactile  stimuli  appeal  both  to 
the  sensory  cortex  and  to  the  optic  thalamus.  For  not  only  is  a  touch 
always  related  to,  and  distinguished  from,  something  that  has  gone 
before  it,  but  we  have  shown  that  contact,  especially  of  an  object 
moving  over  hair-clad  parts,  is  capable  of  exciting  thalamic  activity. 
Vibrations  of  the  tuning-fork  also  make  a  double  appeal,  for  when 
the  cortical  paths  are  cut  the  amplitude  of  the  vibration  must  be 
greater  in  order  that  it  may  be  appreciated;  on  the  other  hand,  the 
vibratory  effect  may  be  stronger  on  the  abnormal  side  in  those  thalamic 
cases  where  the  affective  response  is  excessive. 

But  these  two  centers  of  consciousness  are  not  coequal  and  inde- 
pendent. Under  normal  conditions  the  activity  of  the  thalamic 
center,  though  of  a  different  nature,  is  dominated  by  that  of  the  cortex. 
When  the  sensation  normally  produced  by  a  prick  is  examined  it  is 
recognized  that  the  pain  develops  slowly  and  lasts  a  considerable  time 
after  the  stimulus  has  ceased.  Moreover,  the  same  intensity  of  stimu- 
lation will  produce  a  different  effect  on  the  same  spot  on  different 
occasions.    A  long  latent  period,  persistence  and  want  of  uniformity 


CORTICAL  SENSORY  DISTURBANCES  483 

are  characteristic  of  all  painful  sensations.  This  is  seen  in  an  exag- 
gerated form  in  cases  where  the  thalamic  center  has  been  freed  from 
control.  The  response  to  prick  is  slow,  but  persists  long  after  the 
stimulus  has  ceased.  Moreover,  the  reaction,  when  it  occurs,  tends 
to  be  explosive;  it  is  as  if  a  spark  had  fired  a  magazine  and  the  conse- 
quences are  not  commensurate  with  the  cause. 

On  the  contrary,  the  sensations  normally  produced  by  moderate 
tactile  stimuli  are  characterized  by  a  short  latent  period,  and  disappear 


Figs.  235,  236,  and  237. — Topography  of  the  sensory  disturbance,  in  a  case  of  the 
thalamic  syndrome,  right  side,  of  six  years'  duration  in  a  woman,  aged  fifty-five  years. 
There  is  mild  hemiplegia  of  the  right  side  with  intense  choreo-athetoid  movements 
of  the  hand  and  foot.  There  is  a  marked  ataxia  in  all  voluntary  movements  of  the 
same  side.    Exaggeration  of  the  tendon  reflexes  of  the  right  side.    No  Babinski  reflex. 

There  are  very  active  and  severe  spontaneous  pains  over  the  entire  right  side,  tactile, 
pain,  and  thermal  sensibility  is  diminished,  but  not  abolished,  on  the  entire  right  side. 
Compass  discrimination  markedly  affected.  Complete  astereognosis.  Taste,  smell,  and 
hearing  are  diminished  on  the  right  side.  Vision  is  unimpaired  and  there  is  no  hemi- 
anopsia. Deep  sensibility  is  more  altered  than  superficial.  The  sensory  position  is 
altered,  bony  sensibility  is  much  diminished.     (Thomas  and  Chiray.) 

almost  immediately  on  the  cessation  of  the  stimulus.  A  lesion  of  the 
sensory  cortex  disturbs  both  these  characteristics.  Tactile  sensations 
become  uncertain  and  incalculable,  and  no  threshold  can  be  obtained; 
persistence  and  hallucinations  mar  the  uniformity  of  the  records. 

The  work  of  Head  and  Holmes  tends  to  show  that  the  sensory  cortex 
is  the  organ  by  which  attention  can  be  concentrated  on  any  part  of 
the  body  that  is  stimulated.  The  focus  of  attention  is  arrested  at  any 
one  spot  by  the  changes  produced  by  cortical  activity.     These  are 


484 


DISEASES  OF   THE  BRAIN 


sorted  out  and  brought  into  relation  with  other  sensory  processes, 
past  or  present.  Then  the  focus  of  attention  sweeps  on,  attracted  by 
some  other  object. 

All  stimuli  which  appeal  to  the  thalamic  center  have  a  high  thresh- 
old. They  must  reach  a  high  intensity  before  they  can  enter  conscious- 
ness, but  once  they  have  risen  above  the  threshold  they  tend  to  pro- 
duce a  change  of  excessive  amount  and  duration,  and  this  it  is  the 
business  of  the  cortical  mechanism  to  control.  The  low  intensity  of 
the  stimuli  that  can  arouse  the  sensory  cortex,  and  its  quick  reaction 
period,  enable  it  to  control  the  activity  of  the  cumbersome  mechanism 
of  the  thalamic  center. 


Liiepmami 
(Reg.  rat.) 


Bychowski 


KroU 
V.  StaxtKenberg    (Krykow) 


KroU 

" '    (Iwanow) 


T.  Monakow 
(Vet..r) 

+  +  + 
V.  Bychowski 


Fig.  238. — -Localization  of  apraxia.  Positive  cases  from  lesion  in  the  left  supra- 
marginal  gyrus  and  (a,  b,  c,  d)  small  metastatic  tumors  from  one  case  of  total  bilateral 
apraxia.     In  this  case  foci  were  also  found  in  both  optic  thalami.     (von  Monakow.) 


This  view  of  the  sensory  mechanism  explains  many  of  the  facts 
recognized  by  neurologists  and  psychiatrists.  It  enables  one  to 
understand  how  integrations  can  occur  at  all  afferent  levels  of  the 
nervous  system,  and  makes  development  possible  even  in  the  individ- 
ual. The  aim  of  human  evolution  is  the  domination  of  feeling  and 
instinct  by  discriminative  mental  activities.  This  struggle  on  the 
highest  plane  of  mental  life  is  begun  at  the  lowest  afferent  level,  and 
the  issue  becomes  more  clearly  defined  the  nearer  sensory  impulses 
approach  the  field  of  consciousness. 

Apraxia. — This  term  was  first  used  by  Gogol  in  1873,  in  a  Breslau 
thesis  on  Aphasia.     His  patient  ate  his  soap,  urinated  in  his  water 


APRAXIA  485 

pitcher,  and  was  described  as  having  lost  his  understanding  for  objects. 
Such  defects  had  been  noted  before,  and  it  is  worthy  of  note  that 
Hughlings  Jackson,  in  1866,  called  attention  to  a  similar  type  of 
phenomenon,  and  attached  much  importance  to  it.  Quaglino,  in 
1867,  described  a  case,  Finkelnburg,  in  1870,  another,  in  which 
recognition  of  things  and  people  was  lost,  and  created  the  term  asym- 
bolia.  Wernicke,  in  1874,  expanded  the  term  asymbolia,  while  Freud 
finall}^  utilized  the  term  agnosia,  to  cover  all  types  of  loss  of  sensory 
or  motor  object  images,  apraxia  then  being  arranged  as  a  form  of  loss 
of  knowledge  of  objects,  really  a  form  of  visual  agnosia. 

From  this  early  use  of  the  term  apraxia,  there  has  been  a  distinct 
variation,  brought  prominently  into  the  foreground  by  Liepmann 
in  1900.  He  defined  the  disturbance  as  a  lack  of  knowledge  of  the 
use  of  objects,  although  there  was  no  true  agnosia,  or  loss  of  recog- 
nition of  what  they  were.  Out  of  the  later  studies  of  Liepmann, 
Pick,  von  jMonakow,  d'Hollander  and  others  has  come  the  following 
general  definition  of  apraxia : 

It  consists  in  an  inability  to  perform  certain  subjectively  purposeful 
movements,  or  movement  complexes,  the  motor  power,  sensation, 
and  coordination  being  intact.  Such  an  inability  will  naturally  depend 
upon  at  least  three  factors;  one  may  be  unable  to  recognize  the  object 
which  is  to  be  used,  in  which  case  we  can  speak  of  a  sensory  apraxia, 
in  the  same  sense  as  one  speaks  of  a  sensory  aphasia,  or  a  visual  agnosia. 
Should  the  patient  recognize  the  object,  call  it  perhaps  by  name, 
state  its  use,  and  yet  in  attempting  to  use  it  totally  fail  in  proper 
motor  act,  one  speaks  of  a  motor  apraxia.  It  being  understood  here 
that  there  is  no  necessary  change  in  the  motor  arc,  either  on  the  incoming 
sensory  or  outgoing  motor  side.  Thus  one  can  speak,  as  Wilson  has 
done,  of  a  motor  aphasic,  as  having  an  apraxia  of  his  speech  muscula- 
ture. In  grave  intracerebral  changes  the  knowledge  of  the  proper 
kinetic  images  to  carry  out  purposeful  actions  in  the  arms  and  legs 
may  be  complexly  involved.  Here  one  speaks  of  an  intrapsychic 
apraxia.  Clinicalh'  it  is  usually  overlaid  in  the  general  psychic  loss, 
and  is  often  included  loosely  in  the  term  dementia. 

Apraxia  may  be  then  either  sensory  or  motor;  it  may  be  unilateral 
or  bilateral,  it  may  be  extensive,  involving  many  muscular  groups 
or  may  be  limited  to  a  few,  such  as  an  inability  to  protrude  the  tongue 
on  demand  or  close  the  eyelids,  etc.,  with  perfect  power  in  other 
movements. 

A  certain  patient  with  motor  apraxia,  on  being  given  a  cigarette 
holder  and  cigarette,  recognized  the  objects,  said  they  were  for  smoking, 
but  on  being  told  to  put  them  together  was  unable  to  make  the  correct 
movements,  and  finally  gave  up.  Another  patient  was  given  a  candle 
and  a  match-box.  She  took  out  a  match,  made  rubbing  movements 
with  it  in  the  air  above  the  candle,  and  then  reinserted  it  in  the  box. 

Liepmann's  celebrated  case  was  able  to  do  things  with  his  left  hand, 
but  failed  entirelv  with  his  right.    When  told  to  brush  the  examiner's 


486 


DISEASES  OF   THE  BRAIN 


coat,  he  picked  up  a  corner  of  it  carefully  in  his  left  hand,  then  picked 
up  the  brush  in  his  right  hand,  with  which  he  made  movements  as  if 
to  brush  his  hair.    Asked  to  pour  water  into  a  glass  from  a  carafe, 


Hartmann  III  _ 


Hartmann  I 


V.  Monakovv 
(Elmer) 


Hartmann  II 


_  V.  Monakow 
(Heuss.) 

,    Goldsteitl 


Liepmann  - 


V.  Monakow' 
(Gloor.  b) 


Rhein 


Rhein 


Fig.  239.— Site  of  the  lesion  in  20  cases  of  apraxia  marked  according  to  the  author's 
localizations.  The  shaded  places  in  the  illustration  indicate  the  spreading  of  the  lesion 
in  certain  cases.  Str,  copora  striata;  VH,  anterior  horn  of  ventricle;  HH,  posterior 
horn,     (von  Monakow.) 


he  grasped  the  carafe  with  his  left  hand,  to  pour  water  into  the  glass 
held  in  the  right  hand,  after  which  the  glass  was  brought  to  the  mouth 
without  any  water  in  it.  These  patients  fail  to  carry  out  the  simple 
commands  to  blow  a  kiss,  make  a  threatening  fist,  soldier's  salute,  etc. 


APRAXIA  487 

In  ideomotor  apraxia  the  situation  is  more  complicated.  One 
patient  given  a  tooth-brush  recognized  it,  then  began  to  brush  his 
beard  with  it  chunsily;  another  being  given  a  pistol,  which  he  named 
correctly,  on  being  told  to  shoot  it,  grasped  the  barrel,  blinked  and 
put  the  muzzle  into  his  left  eye.  Another  patient,  being  given  a  cigar 
and  a  match-box  opened  the  latter,  stuck  the  cigar  in  it,  and  tried  to 
shut  the  box  as  though  it  were  a  cigar  cutter.  Then  taking  the  cigar 
out  rubbed  it  on  the  side  of  the  box  as  though  it  were  a  match.  The 
entire  order  of  procedure  was  badly  devised. 

Apraxia  is  usually  an  accompaniment  of  certain  apoplectic  attacks. 
The  lesions  center  about  the  fibers  of  the  corpus  collosum  and  the 
medullary  fiber  areas  from  the  second  parietal  region. 

In  left  hemisphere  disease,  the  apraxia  may  be  homo-  or  hetero- 
lateral,  and  in  homolateral  apraxias  the  corpus  callosum  is  usually 
involved.  In  left  frontal  disease  apraxia  has  been  found  especially 
with  lesions  of  F.l  and  F.2.  Anything  that  brings  about  an  isolation, 
diaschisis,  of  the  left  frontal  area  from  the  right  frontal  area  will 
seem  to  bring  about  an  apraxia. 

Hemianopsia  is  an  infrequent  sign  occurring  as  a  result  of  the 
apoplexy.  As  a  symptom  of  middle  cerebral  disease  it  may  result 
from  thalamic  lesions  (pulvinar,  geniculates)  and  is  usually  a  per- 
manent residual.  It  is  rarely  a  temporary  condition  but  may  show 
marked  improvement.  The  type  is  a  bilateral  homonymous  hemian- 
opsia. Quadrant  hemianopsias  more  often  belong  to  the  middle 
cerebral  syndromes,  although  limited  lesions  of  the  cuneus  may  cause 
quadrant  hemianopsias.^ 

The  visual  fields  will  vary  greatly  according  to  the  tract  involve- 
ment. In  posterior  cerebral  syndromes  occipital  cortical  types  of 
hemianopsia  are  present.  These  show  very  irregular  visual  fields. 
Psychical  blindness  (loss  of  visual  memories)  may  also  result  from 
posterior  cerebral  involvements.  Here  the  portions  of  the  occipital 
cortex  about  the  calcarine  fissure  and  cuneus  are  implicated.  It  is 
usually  an  accompanying  symptom  of  the  apoplectic  attack  but 
may  be  isolated  and  often  combined  with  a  hemianopsia. 

Aphasia  is  a  frequent  result  of  the  hemiplegic  attack  (right  hemi- 
plegia in  right-handed  persons;  left-sided  in  left-handed  persons). 

The  type  of  aphasia  depends  entirely  upon  the  location  of  the 
vessels  and  the  areas  served.  Transitory  aphasias  are  extremely 
common,  occurring  in  at  least  one-half  of  the  attacks.  Permanent 
aphasias  are  rarer,  about  one-half  of  the  patients  recovering.  The 
different  forms  of  aphasia  and  the  localization  significance  are  discussed 
under  Disorders  of  Speech.    (See  p.  237.)^ 

Papillary  edema,  even  choked  disk,  may  be  a  symptom  of  apoplexy, 

1  Compare  Willbrand  and  Saenger,  Die  Neurologie  des  Auges  for  complete  discus- 
sion and  bibliography;  also,  Henschen,  in  Lewandowsky's  Handbuch  der  Neurologie. 

2  Comp.  V.  Monakow's  Die  Lokalisation  im  Grosshirn,  1914,  for  most  recent  discussion 
of  the  complicated  aphasia  question. 


DISEASES  OF   THE  BRAIN 

and  is  found  on  the  side  affected.  It  is  most  frequent  with  large 
lesions.^ 

Diagnosis. — A  separation  of  hemorrhage,  thrombosis,  and  embohsm 
is  highly  desirable,  although  not  always  possible.  The  end-results 
are  identical,  but  the  antecedent  conditions  vary.  Hemorrhage  is 
comparatively  rare  in  the  young,  but  it  may  be  seen  at  all  decades, 
and  even  in  intra-uterine  life.  When  occurring  in  early  youth  it  is 
usually  a  result  of  parental  syphilis  or  some  acute  illness.  In  cases 
of  hemorrhage  the  patient  is  usually  over  forty,  shows  signs  of  arterial 
disease,  frequently  with  hypertrophied  heart,  and  often  nephritis. 
The  attack  is  usually  sudden,  often  preceded  by  emotional  disturb- 
ance or  sudden  change  in  position  that  modifies  the  blood-pressure. 
The  attack  is  more  likely  to  be  accompanied  b}^  unconsciousness 
(75  per  cent.).  Severe  ventricular  hemorrhages  show  blood  on  lumbar 
puncture,  but  this  procedure  is  rarely  called  for  save  in  supposed 
traumatic  cases. 

Thrombosis  occurs  also  in  older  individuals,  and  in  syphilitics  par- 
ticularly. Unconsciousness  is  less  apt  to  occur,  or  occurs  in  stages  as 
it  were.  The  prodromata  already  mentioned  are  more  apt  to  have 
been  present.  Markedly  atheromatous  arteries  speak  for  thrombosis, 
and  mild  signs  of  deterioration — lacunar  syndromes — speak  for 
thrombotic  types  of  disease. 

Embolism  is  almost  invariably  associated  with  some  acute  disease — 
typhoid,  acute  septic  infections  (articular  rheumatism,  gonorrhea, 
scarlet  fever,  malaria,  etc.).  Acute  endocarditis  is  often  present 
and  the  individuals  are  apt  to  be  young.  Unconsciousness  is  less 
apt  to  occur;  when  occurring  it  is  likely  to  be  acute,  and  is  more 
often  present  in  basilar  and  carotid  occlusions  than  when  other  arteries 
are  blocked. 

Hemorrhages,  especially  when  small,  frequently  show  the  maximum 
symptoms  early,  with  gradual  betterment,  while  thromboses  usually 
show  the  reverse,  the  symptoms  having  a  tendency  to  spread  or  to 
deepen. 

Ophthalmoscopic  examination  is  always  imperative.  Dilatation  of 
the  pupil,  usually  present,  can  be  obtained  by  cocain  in  a  few  minutes. 
Diffuse  retinitis,  so-called  retrobulbar  neuritis,  is  highly  indicative  of 
basal  syphilis  or  syphilitic  endarteritis.  In  hemorrhage  the  vessels 
of  the  disk  are  apt  to  be  engorged.  This  is  not  so  in  embolism  nor  in 
thrombosis.    Choked  disk  points  to  a  neoplasm. 

The  blood-pressure  affords  diagnostic  criteria.  It  is  apt  to  be  high 
in  hemorrhage  and  in  embolism,  but  low  in  thrombosis,  also  in 
neoplasms,  syphilitic  endarteritis,  and  cerebral  abscess. 

Other  disease  processes  to  be  distinguished  are  hysterical  hemiple- 
gias, syncope,  epileptic  attacks,  general  paresis,  uremic,  alcoholic,  or 
encephalitic  coma. 

1  Uhthoff,  Neurol.  Centralblatt,   1909. 


DIAGNOSIS  OF  APOPLEXY  489 

Hysterical  hemiplegias  are  rarely  accompanied  by  unconsciousness. 
Hysterical  delirium  may  complicate  the  picture.  Later  the  signs  of 
pyramidal  tract  involvement,  such  as  increased  reflexes,  Babinski, 
Chaddock,  loss  of  abdominal  reflexes,  Grasset  and  Hoover  phenomena 
are  not  present  in  hysterical  hemiplegia.  In  certain  mild  thalamic 
cases  the  sensory  changes  may  be  regarded  as  hysterical,  but  careful 
summing  up  of  the  results  outlined  on  pages  55-63  will  show  the 
involvement  of  the  thalamus.  Personal  experience  has  shown  that 
many  thalamic  cases  are  diagnosed  as  hysterical. 

Certain  lacunar  syndromes,  especially  in  the  anterior  cerebral 
distribution,  which  are  mild  and  which  present  momentary  confusion 
(worse  at  night),  emotional  instability,  irritability,  tendency  to 
weeping,  etc.,  are  frequently  termed  hysteria. 

Lacunar  syndromes  with  softenings  in  the  frontal  areas  are  also 
mistaken  for  manic  attacks.  There  is  confusion,  some  disorientation, 
excitement,  and  after  a  short  time  the  whole  thing  may  clear  up, 
leaving  only  slight  defect,  easily  overlooked,  unless  careful  intelligence 
tests  are  applied  (presbyophrenic  excitements,  presenile  excitements 
— see  Senile  and  Presenile  Psychoses.) 

Uremic  coma  is  usually  of  more  gradual  onset.  There  are  preceding 
signs  of  heaviness  and  toxemia;  convulsive  movements  are  not  infre- 
quent, with  little  signs  of  irregular  respiration  of  the  two  sides  of  the 
chest,  possible  pupillary  inequalities,  possible  variations  in  response 
to  sensory  stimuli  on  the  two  sides,  involuntary  reflex  motor  responses 
to  joint  squeezing  and  finger  squeezing  (Marie-Foix)  are  usually  absent 
in  uremic  coma.  .  The  general  odor  of  the  patient  is  often  characteristic 
in  uremia.    Some  uremic  patients  develop  apoplectic  attacks  as  well. 

Diabetic  coma  shows  similar  difficulties.  Acetone  odor,  large 
amounts  of  sugar-containing  urine,  slow  onset,  and  the  previous 
history  must  be  relied  upon. 

Alcoholic  coma  is  frequently  diagnosed  by  the  police  as  apoplexy, 
since  many  alcoholics  do  have  apoplectic  attacks.  Some  severe 
intoxications  resemble  apoplectic  coma  very  closely,  but,  as  a  rule, 
the  coma  is  less  profound  in  alcoholism;  the  reflexes  are  often  pre- 
served, in  a  measure,  and  the  uniformitj-  in  the  bilaterality  of  the 
relaxation  is  evident  (absence  of  Marie-Foix  signs).  One  should  be  on 
one's  guard,  however,  and  look  for  all  of  the  little  signs  of  hemiplegia.^ 

Ejjileptic  arid  syncopal  attacks  rarely  present  great  difficulties. 
The  history  of  a  previous  attack,  the  scarred  bod}^  or  head  and  tongue 
of  the  epileptic  is  often  evident,  while  in  syncope  the  coma  is  usually 
shallow  and  the  feeble  respirations  and  superficial  heart  action  point 
to  the  difficulty. 

An  apoplectiform  attack  may  be  the  first  sign  of  paresis.  It  is 
a  cerebral  edema,  and  may  be  at  first  indistinguishable  from  an 
apoplexy  (non-paretic) .    The  later  history  and  examination  will  usually 

1  Dejerine,  Semiologie,  1914,  2d  ed. ;  Jelliffe,  Little  Signs  of  Hemiplegia,  Postgraduate, 
1912. 


490  DISEASES  OF  THE  BRAIN 

establish  a  diagnosis,  although  the  pseudoparesis  of  arteriosclerotic 
softening  is  often  only  distinguishable  from  paresis  by  the  cytobio- 
logical  four  reactions.  (See  chapter  on  Syphilitic  Diseases  of  the 
Nervous  System,  p.  511.) 

Prognosis. — Recovery  from  the  attack  and  amelioration  of  the  residual 
symptoms  are  separate  problems. 

Cerebral  hemorrhage  is  usually  more  immediately  fatal  than  either 
thrombosis  or  embolism.  Deepening  coma,  Cheyne-Stokes'  respira- 
tion, irritative  phenomena,  jerking,  convulsions  (very  high,  over 
230  mm.  or  very  low,  under  90  mm),  blood-pressure,  marked  rise 
in  temperature  are  the  usual  lethal  signs.  General  convulsions, 
retinal  hemorrhages,  blood  on  puncture,  bilateral  paralysis  are  unfavor- 
able signs.  After  recovery  from  coma,  continued  temperature,  advanc- 
ing symptoms,  restlessness,  delirium,  loss  of  sphincter  control,  trophic 
disturbances,  indicate  a  grave  prognosis  and  probable  death  in  from 
two  to  three  weeks. 

Early  attacks  of  lacunar  softening  are  rarely  fatal,  but  indicate 
that  a  fatal  termination  from  a  more  severe  type  of  attack  will  be 
probable  in  from  one  to  three  years.  Inasmuch  as  this  is  a  form  which 
is  usually  called  hysterical  in  the  early  mild  attacks,  sometimes 
diagnosed  as  a  mild  manic  attack,  from  the  excitement  and  confusion, 
one  should  be  on  one's  guard  in  this  not  infrequent  syndrome. 

The  recovery  from  the  residual  symptoms  will  vary  greatly  upon 
the  nature,  localization,  and  extent  of  the  lesion.  A  careful  plotting 
of  the  entire  symptomatology  will  determine  the  area  involved  in  the 
destructive  process,  and  one  must  first  separate  out  the  effects  of 
diaschisis  from  those  of  actual  tissue  destruction,  since  the  former 
are  more  apt  to  disappear. 

Aphasias  are  usually  recovered  from  almost  invariably  in  left- 
sided  hemiplegias  in  the  right-handed.  In  right-handed  hemiplegias 
aphasias  are  usually  recovered  from  in  about  one-half  of  the  patients, 
especially  in  the  intelligent,  who  will  make  an  effort  to  reeducate 
themselves.  If  the  lesion  lies  directly  within  the  aphasic  area  the 
chances  for  recovery  are  less. 

Hemianopsias  are  not  infrequently  diaschitic.  When  so  they  pass 
within  a  few  weeks.  In  thalamus  lesions  they  are  apt  to  be  permanent, 
and  cortical  hemianopsias  persisting  over  a  few  weeks  are  apt  to  be 
permanent. 

Hemiplegia,  if  total,  is  apt  to  persist  in  some  degree  at  least,  although 
most  patients  are  able  to  get  about  in  three  months  or  more.  Help- 
lessness persisting  over  six  months  or  a  year  has  been  partially 
recovered  from.  Facial  palsies  usually  recover.  Leg  palsy  is  rarely 
as  persistent  as  arm  palsy,  but  both  react  favorably  to  appropriate 
treatment.    Early  contracture  is  a  bad  prognostic  feature. 

Thalamic  involvement  is  a  bad  prognostic  feature— the  pain  and 
irregular  movements  (choreo-athetoid),  hemianesthesia,  etc.,  usually 
persist. 


TREATMENT  OF  APOPLEXY  491 

Mental  defects  may  clear  up  almost  entirely,  especially  when  slight, 
but  careful  intelligence  tests  (see  chapter  on  Mental  Examination) 
should  be  utilized  in  all  cases,  especially  to  decide  medicolegal  prob- 
lems which  may  arise — testamentary  or  contract  capacity,  respon- 
sibility, etc.  A  sensory  or  motor  aphasia  alone  is  not  necessarily  a 
sign  of  an  intelligence  defect;  many  aphasics  are  very  intelligent. 
They  simply  cannot  utilize,  in  speech,  the  knowledge  they  have. 
(See  chapter  on  Senile  and  Presenile  Mental  States.)  A  motor  aphasic 
who  cannot  get  his  symbols  over,  as  it  were,  may  not  be  any  more  of 
a  dement  than  an  Englishman  trying  to  make  a  Chinaman  understand 
what  he  is  saying. 

Treatment. — Prophylaxis  applies  particularly  to  those  over  fifty 
years  with  arteriosclerosis,  and  sustained  high  blood-pressure.  The 
tendency  to  hemorrhage  here  is  great.  Such  patients  should  slow  down 
somewhat  in  their  work,  if  strenuous  and  calling  for  intense  and  sus- 
tained effort,  and  especially  if  emotional  calls  are  frequent.  A  moderate 
amount  of  work  and  freedom  is  desirable.  Invaliding  a  prospective 
hemorrhagic  case  is  inadvisable. 

Dietary  faddism  is  to  be  avoided.  Excessive  eating  is  to  be  avoided 
and  all  alcohol  should  be  restricted.  Protein-free  diet — vegetable 
proteins  are  the  same  as  animal — keeps  down  the  blood-pressure  in 
many  cases.  Some  search  should  be  made  to  see  if  specific  protein 
sensitization  exists. 

Careful  regulation  of  the  gastro-intestinal  tract  is  called  for,  the 
kidney  functions  should  be  scrutinized,  and  the  liver  metabolism 
regulated. 

It  is  doubtful  if  drug  therapy  is  of  any  service  in  prophylaxis.  The 
iodides  have  been  used  widely,  but  their  utility  is  still  undecided. 

Treatment  of  the  attack,  even  if  there  is  no  unconsciousness,  requires 
immediate  rest  in  bed  if  possible.  If  stricken  away  from  home  the 
patient  should  be  moved  as  little  as  possible.  With  high  tension,  hot 
foot-packs  will  help  to  reduce  it  (not  hot  enough  to  burn  the  unconscious 
patient).  In  cases  in  which  the  blood-pressure  rises  steadily,  keeps 
above  250  mm.  and  with  very  deep  coma,  blood-letting  (10  to  12  oz.) 
is  advisable. 

Hypodermic  medication  by  blood-pressure  reducing  drugs  in  hemor- 
rhage is  alone  advisable  in  coma,  and  only  very  small  quantities  of 
water  should  be  used,  as  water  raises  blood-pressure.  Hydrochlorate 
of  gelsemine  in  doses  of  ^w  grain  is  fairly  active  and  reliable.  The 
nitrites  are  not  available. 

If  the  patient  can  swallow,  tincture  of  aconite  in  TTlv  doses  may  be 
given,  watching  the  blood-pressure.  The  dose  may  be  repeated  in  an 
hour.  Pressure  should  be  kept  below  200  mm.  if  possible.  Tincture 
of  veratrum  viride  inTTlv-xv,  every  two  hours,  or  the  fl.  ext.  of  gelsem- 
ium  in  same  doses  at  same  intervals. 

Pressure-reducing  drugs  should  be  used  with  caution.  The  high 
pressure  following  hemorrhage  is  usually  compensatory  and  for  the 


492  DISEASES  OF   THE  BRAIN 

purpose  of  keeping  up  an  effective  vascular  irrigation  of  the  medullary 
nuclei  following  a  rupture  in  the  arterial  pipe  line.  These  drugs  should 
be  used  only  when  it  is  known  that  a  high  pressure  preceded  the  attack. 

Early  purgation  is  desirable;  2  gtt.  of  croton  oil  is  useful  in  states 
of  deep  coma.  This  may  be  placed  with  butter  on  the  back  of  the 
tongue. 

If  there  is  marked  excitement,  chloral,  gr.  v-xv,  or  paraldehyde, 
5j-ij,  may  be  given  by  mouth  or  by  rectum.  Tepid  sponge  baths 
help  restlessness.  Veronal  and  trional  are  useful  in  the  restlessness 
of  lacunar  softenings. 

Care  of  the  mouth,  drooling,  moving  the  patient,  removal  of  all 
obstructions  to  breathing,  should  be  attended  to  at  once. 

Nourishment  may  be  omitted  in  the  early  stages.  A  purin-free  diet, 
mostly  milk  and  gruels,  to  which  sugar  and  eggs  may  be  added  later, 
should  constitute  the  standard  diet.  In  the  presence  of  dysphagia, 
milk  enemas  are  to  be  given. 

In  cardiac  atonic  cases,  with  low  blood-pressure — chiefly  thromboses 
— vasodilators,  camphor,  and  alcoholic  stimulants  may  be  necessary. 

Surgical  treatment  may  be  advisable  for  meningeal  or  subcortical 
hemorrhages.  Certain  nephritic  cases  are  helped  by  lumbar  puncture, 
and  the  withdrawal  of  20  to  50  c.c.  of  fluid.  The  patients  should  be 
kept  quiet  several  weeks,  attention  being  paid  to  giving  ease  and 
comfort  by  cushions,  props,  supports,  and  frequent  changes  of  position. 

The  late  treatment  should  be  gradually  adapted  to  the  residual 
symptoms.  The  gastro-intestinal  tract  and  the  skin  need  special 
attention.  The  care  of  the  paralyzed  muscles  by  systematic  exercises, 
the  avoidance  of  contractures  and  treatment  or  shoulder,  of  hip,  or 
knee  arthritides  constitute  the  major  portion  of  the  late  therapy. 

Electricity  is  of  doubtful  service,  save  as  a  psychic  aid  to  the  general 
management  of  the  case.  Galvanism  aids  some  of  the  pains,  the  anode 
should  be  over  the  painful  area. 

The  general  care  of  the  invalid  will  depend  largely  upon  his  economic 
status.  Travel  is  helpful  in  supplying  a  mental  stimulus  and  the 
warmer  climates  and  more  interesting  foreign  resorts  are  enjoyable, 
and  thus  of  direct  therapeutic  value.  Varied  occupations  suited  to 
the  individual's  temperament  and  habits  should  be  sought  for,  wherein 
resourcefulness  is  a  great  asset. 

Speech  training  for  aphasia  may  accomplish  much.  Its  details, 
as  well  as  many  others,  cannot  be  entered  into  here.^ 

1  Consult  White  and  Jelliffe,  Modern  Treatment  of  Nervous  and  Mental  Disease, 
vol.  ii,  for  detailed  discussion  of  all  of  the  features  of  treatment  in  articles  by  F.  Tilney 
and  S.  A.  K.  Wilson. 


CHAPTER   XIV. 
TUMORS  OF  THE  BRAIN. 

Tumors  of  the  brain  are  relatively  infrequent.  They  occur  at  all 
ages,  are  found  in  every  conceivable  location,  and  are  of  a  greatly 
varied  pathology.  Extensive  monographs  have  been  published,  and 
no  feature  of  nervous  disease  has  attracted  more  attention,  not  only 
from  its  practical  importance,  but  also  from  the  stand-point  of  cerebral 
localization  and  function.  The  chief  literature  to  1915  may  be  found 
in  Starr,^  von  Monakow,^  Oppenheim,^  Tooth,^  and  Redlich.'^ 

In  18,000  cases  of  nervous  disease  occurring  in  ten  years  at  the 
Vanderbilt  Clinic  there  were  48  brain  tumors.  This  is  a  minimum 
computation.^  Bruns  states  it  as  high  as  2  per  cent.,  Cushing  as 
0.75  per  cent.,  Redlich,  from  4000  cases  of  nervous  disease,  the  same. 

Etiology.^The  causes  for  certain  tumors,  such  as  tuberculoma, 
syphiloma,  actinomycoses,  are  well  known.  For  both  syphilitic  and 
tuberculous  tumors,  and  possibly  other  tumors,  traumatism  may  be 
an  additional  element  for  a  brain  localization.  Metastatic  tumors 
follow  from  their  primary  sources.  Certain  teratomata,  dermoids, 
angiomata  are  congenital  conditions,  while  cholesteomata,  chordoma, 
chondroma,  lipoma,  and  myxoma  are  also  developmental  anomalies. 

The  traumatic  genesis  of  tumors  in  general,  or  of  any  one  type  in 
particular,  apart  from  aneurisms,  is  problematical,  yet  if  the  trauma 
and  symptoms  are  related  in  special  waj^s  the  causative  role  may  be 
assumed.  Thus  if  the  injury  is  sufficiently  intense  to  definitely  injure 
the  skull,  the  time  interval  between  the  accident  and  the  development 
of  the  symptoms  not  too  great,  and  the  localization  of  the  probable 
tumor  near  to  the  site  of  the  injury,  the  relationship  may  be  justifiably 
maintained. 

Varieties. — The  chief  forms  met  with  are  (1)  true  tumors,  (2)  infec- 
tious tumors,  (3)  parasitic  cysts,  (4)  aneurisms,  (5)  vascular  cysts. 

1.  True  Tumors. — Of  these  gliomata  are  the  most  frequent.  They 
preponderate  over  any  other  class  in  adults.  In  Tooth's  summary  of 
500  cases,  49  (2  per  cent.)  were  gliomata.  Children  rarely  come  to 
the  National  Hospital  in  London,  hence  his  low  percentage  of  tuber- 
culous tumors.  Gliomata  occur  throughout  the  brain  as  circumscribed 
or  as  diffuse  tumors,  and  are  extra-  as  well  as  intracerebral.    The 

1  Textbook  of  Nervous  Diseases,  4th  ed.,  Brain  Surgery. 

2  Gehirnpathologie,  2nd  ed.,  Die  Lokalisation  im  Grosshirn,  1914. 

3  Die  Geschwiilste  des  Gehirns,  2nd  ed.  *  Brain,  1912. 
*  Handbuch  der  Neurologie,  Lewandowsky,  vol.  iii,  1912. 

6  See  Report  of  Clinic  of  Prof.  M.  Allen  Starr,  1900-1909. 


494  TUMORS  OF   THE  BRAIN 

general  tendency  is  toward  diffuseness  and  principally  in  intracerebral 
growths  degeneration  with  hemorrhage  and  fatty  and  cystic  formations 
takes  place.  Thus  the  symptoms  are  apt  to  develop  slowly  and  inter- 
mittently in  cortex  or  basal  ganglia,  occasionally  from  the  ependyma. 
They  may  invade  an  entire  hemisphere. 

Sarcomata  are  less  frequent,  restricing  the  term  sarcoma  more 
strictly  than  is  usual,  and  rejecting  the  compromise  gliosarcoma. 
Nearly  all  gliomata  show  elements  indistinguishable  from  sarcoma. 
They  usually  develop  slowly  in  the  brain. 

Chloromata  are  leukemic  in  origin  and  are  rare.  They  involve  the 
periosteum  or  the  base  and  thus  cause  compression  phenomena  which 
often  persist  for  some  time. 

Fihroviata  (neurofibromata)  are  comparatively  common  brain 
tumors   (10  per  cent,  in  Tooth's  collection,   including  fibroglioma) . 


Fig.  240. — Infiltrating  glioma  of  basal  ganglia. 

They  develop  chiefly  about  the  cerebellopontine  angle  (acoustic)  but 
may  develop  along  other  cranial  nerves.  Occasionally,  they  are 
multiple.    They  develop  slowly. 

Endotheliomata  are  comparatively  frequent  (14  per  cent,  in  Tooth's 
collection).  They  seem  to  confine  themselves  chiefly  to  the  anterior 
fossae.  They  are  usually  small  and  multiple,  develop  slowly  and 
chiefly  in  the  falx  region. 

Chordomata  are  infrequent,  and  only  rarely  reach  a  considerable  size.^ 

Carcinomata  (5.8  per  cent. — Tooth),  closely  related  to  the  endothelio- 
mata are  usually  secondary  (metastatic),  rarely  primary. 

Psammomata,  chordomata,  lipomata,  enchondromata,  angiomata, 
osteomata,  adenomata,  cholesteomata,  teratomata  (pineal)  ,2  and 
dermoids  are  among  the  rarities. 

'  Jelliffe  and  Larkin,  Journal  of  Nervous  and  Mental  Disease,  January,  1912. 

2  Bailey  and  Jelliffe,  Tumors  of  the  Pineal  Body,  Arch  f.  Int.  Med.,  December,  1912. 


ANEURISMAL  TUMORS  495 

2.  Infectious  Tumors. — These  are  tuberculous,  syphilitic,  actino- 
mycotic. 

Tuberculomata  are  possibly  the  commonest  of  all  tumors,  certainly 
in  children  (Starr,  193  in  600  tumors  recorded).  They  are  extremely 
rare  after  forty  years.  They  are  frequently  conglomerate  or  multiple 
in  type,  hence  giving  rise  to  mixed  syndromes.  There  may  be  a  few 
very  small  miliary  tubercles  or  a  large  broken-down  tubercle  mass,  with 
every  conceivable  intermediary  stage.  They  develop  chiefly  in  the 
cerebellum,  peduncle,  basal  ganglia,  pons,  and  cortex.  In  Zappert's 
group  of  89,  37  were  in  the  cerebellum,  29  in  the  cerebral  cortex,  13 
in  the  basal  ganglia,  5  in  the  pons.    They  have  a  bad  prognosis. 


/ 

/ 
/ 


Fig.  241. — -Chordoma  of  base.     (Jelliffe  and  Larkin.) 

Syphilomata. — Gummata  are  not  infrequent.  They  are  practically 
limited  to  adults,  and  are  not  recorded  from  congenital  syphilis.  They 
may  appear  from  one  year  to  thirty  years  after  infection.  They  occur 
chiefly  as  fiat,  infiltrating,  irregular  masses — less  often  as  definite 
nodular  masses,  chiefly  at  the  base  of  the  brain.  They  are  discussed 
more  fully  in  the  chapter  on  Syphilis  of  the  Nervous  System  (g.  v.). 

Actinomycosis  of  the  brain  is  a  rarity. 

3.  Parasitic  Cystic  Tumors. — Cysticercus  of  the  brain  is  a  rarity  and 
is  secondary. 

4,  Aneurismal  Tumors. — Aneurisms  are  very  frequent  in  cerebral 
vessels.  They  are  mostly  small,  but  large  aneurisms  occur  at  times 
and  give  symptoms  of  pressure.    They  occur  in  patients  usually  from 


496 


TUMORS  OF   THE  BRAIN 


forty  to  seventy  years  old,  and  are  mostly  of  the  basilar.  They  cause 
pressure  symptoms  at  times,  with  obstructive  symptoms — basilar  syn- 
drome— or  they  rupture  and  produce  symptoms  of  cerebral  hemorrhage.^ 
Symptoms. — Brain  tumors,  even  of  a  large  size,  may  be  found  at 
autopsy,  and  yet  not  have  given  rise  to  any  recognized  symptoms. 
Tuberculomata  are  thus  frequently  found  in  children.  With  more 
precise  investigation  such  latent  tumors  are  becoming  rarer,  especially 
since  the  importance  of  mental  symptoms — psychoses,  so-called  hys- 
terias, etc. — unaccompanied  by  sensorimotor  syndromes,  is  becoming 
recognized.  Many  small  tumors,  especially  osteomata,  psammoma, 
slowly  developing  and  circumscribed  gliomata,  choleosteomata  cause 
very  few  symptoms.  Occasionally  a  tumor  will  show  monosymp- 
tomatically,  as  by  epileptic  convulsions,  mild  speech  disturbances,  mild 
sensory  defects,  optic,  olfactory,  auditory  hallucinations  or  hemian- 
opsia, without  being  recognized. 


Fig.  242. — Gumma  of  brain. 


The  symptoms  are  best  considered  as  (1)  general  and  (2)  local  or 
focal. 

The  general  symptoms  are  indicative  of  the  effects  of  the  tumor 
as  a  whole,  irrespective  of  its  special  nature  or  localization.  They  are 
due  in  general  to  the  effects  of  increased  intracranial  pressure,  which 
in  certain  tumors,  notably  of  the  posterior  fossa  and  cerebellum,  appears 
early  and  is  usually  marked  even  with  small  tumors,  whereas  tumors 
elsewhere  often  may  show  little  of  such  pressure  symptoms.  Some- 
times the  focal  symptoms  appear  before  the  general  ones.  General 
symptoms  rarely  have  any  localizing  diagnostic  value  as  many  of  them 
may  lie  remote  from  the  site  of  the  tumors.    Some  of  the  general  symp- 

1  Beadles,  Brain,  1907,  p.  285;  Reinhardt,  Ueber  Hirnarterienaneurysmen  und  ihre 
Folgen,  Mitt.  aid.  Grenzg.  d.  Med.  u.  Ch.,  1913,  xxvi. 


GENERAL  SYMPTOMS 


497 


toms  of  pressure,  cranial  nerve  palsies  for  example,  may  even  tend  to 
mislead  one  as  to  a  localizing  diagnosis.  Furthermore,  in  cerebral 
tumors,  acute  swellings,  not  identical  with  but  related  to  edemas, 
often  give  rise  to  very  anomalous  symptoms  and  tend  to  obscure  the 
diagnosis  and  render  it  uncertain. 

The  chief  symptoms  of  general  value  are  headache,  nausea,  vomiting, 
dizziness,  respiratory  and  cardiac  disturbances,  metabolic  changes, 
mental  signs  with  sleeplessness — sometimes  drowsiness  and  optic 
nerve  changes,  and  convulsive  phenomena.  These  general  symptoms 
have  a  tendency  to  be  progressive,  but  may  vary  considerably  in  their 
intensity  from  time  to  time,  especially  in  syphilomata,  tuberculomata. 


Fig.  243. — Aneurism  of  basilar  artery.     (Larkin.) 


and  gliomata.     At  times  they  remain  stationary,  again  they  may 
regress  and  disappear. 

Headache. — This  is  frequent.  Most  patients  will  have  headache, 
especially  if  the  tumor  is  of  protracted  growth.  Headache  is  an  early 
sign.  Practically  every  patient  with  a  headache  should  be  questioned 
and  examined  for  the  possibility  of  its  being  caused  by  a  cerebral 
tumor.  The  headache  usually  starts  more  or  less  irregularly,  is  fre- 
quently intermittent  in  the  early  stages  and  then  becomes  persistent, 
being  dull  or  severe;  if  dull,  with  periods  of  excruciating  exacerbation. 
Diurnal  variations  may  be  noted,  and  emotional  excitement  or  general 
causes  for  increasing  intracranial  pressure  increase  it,  often  with  addi- 
32 


498  TUMORS  OF   THE  BRAIN 

tional  symptoms,  such  as  vertigo,  vomiting  or  even  agitated  or  coma- 
tose confusions.  Not  infrequently  aneurismal  tumors  will  reveal 
pulsation  by  auscultation.  Migraine-like  headaches  are  very  frequent 
in  early  stages,  particularly  in  basal  cases,  hypophysis,  basal  gummata, 
neurofibromata,  or  there  may  be  migraine-like  exacerbations  on  a 
dull,  heavy,  gray  background  of  pain.  Children  usually  respond  to 
such  variations  by  attacks  of  screaming,  pulling  the  hair,  or  beating 
the  head. 

Later,  somewhat  mentally  dulled  patients  may  even  deny  any 
headache,  or  even  forget  having  had  a  period  of  great  distress. 

The  headache  of  cerebral  tumor  is  mostly  diffuse,  but  it  may  be 
localized,  in  which  case  it  may  serve  to  indicate  the  general  site  of  the 
tumor.  Such  is  rendered  more  probable  if  further  substantiated  by 
percussion  tenderness,  a  highly  important  procedure,  percussion  dul- 
ness,  and  a:;-ray  shadow.  The  site  of  a  headache  is  a  very  uncertain 
guide  for  localization  purposes,  however.  Frontal  tumors  often  give 
rise  to  occipital  headaches  and  vice  versa;  right-sided  tumors  to  left- 
sided  pains  and  vice  versa.  The  general  drift  for  localized  pain  is,  how- 
ever, in  favor  of  a  similarly  located  tumor.  Among  head  symptoms 
may  be  mentioned  the  occasionally  found  auscultation  notes  of  aneu- 
rismal tumors;  the  presence  of  enlarged  head  (hydrocephalus),  and  the 
overfilling  and  increased  tortuosity  of  the  veins  on  the  forehead,  face, 
conjunctiva,  etc.,  of  the  affected  side. 

Trigeminal  neuralgic  attacks  may  be  general  or  at  times  a  focal 
(cerebellopontine  angle)  sign. 

Nausea,  vomiting,  and  dizziness  are  frequent  in  late  stages  of  a  cere- 
bral tumor,  and  more  often  found  in  children  and  in  those  patients 
with  rapidly  increasing  signs  of  intracranial  pressure — posterior  fossa 
tumors  particularly.  Such  vomiting  may  occur  spontaneously — pro- 
jectile in  type — or  as  an  accompaniment  of  the  headache  crises, 
especially  when  migrainous  in  type.  Vomiting  often  is  absent  entirely 
even  with  large  tumors.  When  present  it  more  often  occurs  in  the 
morning,  and  at  times  it  is  so  persistent  as  to  lead  to  inanition, 
exhaustion,  and  death. 

In  cerebellar,  peduncle,  pontine,  and  medulla  tumors,  and  those 
causing  pressure  on  the  superior  cerebellar  peduncle,  or  its  incoming 
pathways,  the  nausea  and  vomiting  may  be  accompanied  by  unilateral 
vertigoes  or  with  tendencies  to  turn  or  to  fall  in  one  direction.  Here 
general  and  focal  symptoms  coincide.  Frontal  tumors  may  occasion- 
ally cause  such  one-sided  vertigoes  from  implication  of  the  frontal  ex- 
tensions of  the  cerebellar  pathways.  Vertigoes  from  ocular  palsies  are 
localizing  symptoms  (quadrigeminal  syndromes — occasionally  cortical). 

Cardiac  and  Resyiratory  Signs. — Slowness  of  the  pulse,  at  times 
marked — 30  to  40 — is  a  general  sign  of  intracranial  pressure,  and  more 
especially  in  marked  grades.  Hence  it  is  apt  to  be  a  late  rather  than 
an  early  symptom,  unless  one  of  direct  irritation  of  the  vagus  (medulla 
pressure).    The  bradycardia,  at  times  arrhythmia,  may  appear  period- 


GENERAL  SYMPTOMS  499 

ically  during  headache  exacerbations,  or  at  times  independent  of  the 
same  (acute  swelHng  reaction). 

Medulla  tumors  cause  respiratory  changes,  at  times  slowness, 
again  irregularity,  and  Cheyne-Stokes — with  acute  pressure  symptoms 
(hydrocephalus  internus) .  Hiccough,  yawning,  and  related  respiratory 
signs  are  occasionally  present. 

Metabolic  Disturbance.— These  are  irregular  in  their  development 
and  evolution.  Fever  is  infrequent  save  as  a  complication  of  the 
late  stages. 

Cachexia  and  marasmus  are  present  with  certain  carcinomata,  and 
marked  adiposity.  Ovarian,  and  in  particular  testicular  aplasia,  are 
frequent  in  certain  hypophyseal  (pituitary)  tumors  or  those  causing 
internal  hydrocephalus  by  possible  implication  of  the  infundibular 
region  through  general  pressure  (pineal  and  corpora  quadrigemina 
tumors).  Acromegaly  is  a  special  case,  as  are  also  the  dystrophia 
adiposogenitalis  syndromes  which  are  discussed  in  the  chapter  on  the 
disorders  of  the  endocrinous  glands. 

Mental  Signs. — These  are  of  great  value,  both  general  and  localizing, 
in  from  60  to  85  per  cent,  of  the  cases. ^  They  vary  considerably, 
and  are  particularly  prominent  late  in  the  disease,  although  here 
masked  under  the  general  symptoms  of  apathy,  confusion  or  coma. 
Tumors  of  any  region,  large  and  small,  and  independently  of  their 
pathological  nature,  may  cause  psychical  changes.  Certain  localities 
cause  special  psychical  alterations  to  be  discussed  under  focal  and 
localizing  symptoms. 

In  the  early  stages,  slight  impairment  of  attention,  with  slowness 
and  difficulty  in  grasp,  retardation  in  motor  response,  and  a  confusion 
or  bewilderment  may  be  present.  Ready  forgetting,  slight  esthetic 
lapses,  and  moral  breaks — with  the  telling  of  shady  stories,  showing 
of  bad  taste,  exhibitionistic  fancies,  even  gross  lapses — such  as  open 
masturbation,  etc. — occur.  These  are  the  precursors  of  a  more  marked 
grade  of  retardation  of  mental  function,  leading  to  apathy,  listlessness, 
lack  of  initiative,  at  times  with  confusional  episodes — getting  lost — 
fugues,  fussiness,  emotionalism,  etc.  In  marked  states  of  confusion 
and  disorientation  a  typical  Korsakow's  syndrome  (q.  v.)  without 
polyneuritis,  will  show  itself. 

Later  stages  show  typical  pictures,  not  distinguishable  from  those 
of  arteriosclerotic  dementia,  or  paresis — i.  e.,  so  far  as  the  purely 
mental  picture  is  concerned. 

Certain  less  general  symptoms  often  present  themselves  and  are 
of  a  certain  localizing  value.  Hallucinations  of  smell  speak  for  olfac- 
tory lobe  or  olfactory  tract  involvement.  Those  of  sight  for  occipital 
cortex  localization,  those  of  sound  for  temporal  lobe  trouble. 

The  tendency  to  joke,  be  facetious,  show  manic-idea  associations, 
even  flight  of  ideas  with  euphoria,  is  at  times  present.     This  is  more 

1  Gianelli,  Schuster,  and  others.  See  Bibliography  in  Redlich,  Hirntumor,  Lewan- 
dowsky's  Handbuch  der  Neurologie. 


500  TUMORS  OF  THE  BRAIN 

often  found  in  prefrontal  tumors,  especially  left-sided,  but  may  occur  in 
tumors  of  other  regions,  usually,  however,  all  reaching  to  and  involving 
the  cortex.  These  symptoms  are  possibly  thalamic  over-responses 
from  thalamocortical  interference  at  cortical  levels. 

Certain  patients  show  definite  depressed  states — with  hypochon- 
driasis or  even  melancholic  suicidal  ideas.  Others  show  manic  pictures 
with  wild  flight  or  marked  maniacal  delirium.  Certain  patients 
develop  delirium  during  certain  of  their  headache  paroxysms.  Para- 
noid trends  also  manifest  themselves  in  a  few  instances.  So-called 
hysterical  symptoms  are  frequently  encountered.  Careful  analysis 
shows,  however,  no  psychical  conversions.  Emotionalism  and  delirium 
are  incorrectl}'^  termed  hj^sterical  because  of  a  loose  application  of  the 
term  hysteria. 

Optic  Nerve  Changes. — These  are  of  the  greatest  importance  in  diag- 
nosis of  brain  tumor.  The  general  features  are  discussed  in  the  chapter 
on  Cranial  Nerves.  From  60  to  80  per  cent,  of  all  patients  show  optic 
nerve  changes,  which  vary  largely,  depending  upon  the  grade  of  intra- 
cranial pressure  and  the  size  and  location  of  the  tumor.  Those  tumors 
causing  great  intracranial  pressure  (posterior  fossa  particularly) 
naturally  cause  choked  disk  and  optic  neuritis  earlier  and  in  more 
marked  degree.  Optic  nerve  changes  may  be  absent  even  with  large 
tumors,  and  small  tumors  of  the  pons,  medulla,  motor  area,  basal 
ganglia,  corpus  callosum,  and  hypophysis  may  give  rise  to  no  optic  nerve 
changes.  The  optic  nerve  changes  develop  gradually.  They  are 
unilateral,  later  bilateral  or  develop  bilaterally  synchronously.  As  a 
rule  tumors  of  one  side  show  beginning  nerve  changes  on  the  same 
side  earlier  and  more  markedly  than  on  the  opposite  side.  The  reverse 
can  also  be  true  and  the  localizing  value  must  not  be  overestimated. 
The  general  trend,  however,  is  as  stated  (Gunn). 

The  visual  power  may  not  be  lost  even  with  marked  grade  of  swelling 
or  of  atrophy,  but  there  is  later  a  gradual  loss  of  vision — often  seen 
in  early  signs  by  the  irregularity  of  the  color  field  loss  (interlacing 
phenomena,  scotomata,  etc.).  The  hemianopic  changes  which  are  not 
infrequent  in  chiasm,  posterior  tract,  pulvinar,  and  parts  of  the  path- 
ways are  discussed  in  Chapter  V,  p.  183. 

Motor  Phenomena. — These  are  local  or  general.  Epileptiform  attacks 
are  frequent — particularly  in  children.  When  limited  Jacksonian 
attacks  are  present,  the  localizing  value  in  the  motor  cortex  is  evident, 
save  for  the  few  exceptions  of  pontine,  cerebellar,  and  peduncular 
Jacksonian  attacks.  Petit  mal  attacks  are  also  not  infrequent.  Some 
patients  die  in  the  convulsive  seizures. 

Focal  or  Local  Symptoms. — These  may  be  among  the  first  of  the 
symptoms  encountered,  especially  in  circumscribed  cortical  tumors, 
but,  as  a  rule,  the  focal  symptoms  develop  after  the  general  ones;  again 
they  advance  together.  They  are  best  considered  with  reference  to 
the  areas  involved,  such  as  the  sj^mptoms  of  the  frontal  localizations, 
central  convolutions,  parietal  lobes,  temporal,  etc. 


LOCAL  SYMPTOMS— FRONTAL  LOBE 


501 


Frontal  lube  tinnor.s  are  those  located  ahead  of  the  precentral  sulci. 
Those  of  the  frontal  poles,  foot  of  third  frontals,  are  frequently  termed 
prefrontal  tumors.  The  functions  of  the  frontal  areas  are  chiefly 
those  of  coordination  of  psychical,  chiefly  intellectual  processes.^  They 
contain  motor  areas  for  the  innervation  of  the  muscles  of  the  neck, 
throat  and  abdominal  muscles  and  the  third  frontal  convolution — 
Brocas'  convolution — is  the  motor  aphasia  area,  ]Marie  and  his  pupils 
notwithstanding.  On  the  base  the  olfactory  and  optic  pathways  may 
be  involved  by  direct  or  indirect  injury,  and  pressure  posteriorly  causes 
pyramidal  tract  symptoms.  Cerebellar  pathway  projections  also  lie 
in  the  frontal  poles. 

Thus  the  symptoms  of  tumors  lying  within  the  frontal  lobes  may 
show  considerable  variation  according  to  their  size  and  site.     Many 


Fig.  244. — Frontal  lobe  tumor.      (Larkin.) 

small  tumors  located  deep  in  the  cortex  are  apparently  symptomless 
from  the  neurological  point  of  view.  Paranoid  states,  occasionally 
seen  from  such  tumors,  are  readily  overlooked,  also  mild  depressed 
states  which  are  called  neurasthenia.  One  symptom  of  special  impor- 
tance is  a  tendency  to  make  jokes  (Witzelzucht),  or  a  tendency  to 
talk  or  answer  beside  the  point — at  times  an  apparently  intentional 
eft'ort  to  mislead.  One  does  not  necessarily  locate  a  tumor  in  the  frontal 
lobes  by  reason  of  this  tendency  to  joking  alone.  At  times  the  behavior 
is  infantile  and  childish,  and  diagnosed  hysteria.  Again  patients  are 
irritable,  excitable,  churlish,  even  have  furious  outbreaks  of  wrath  and 
are  violent,  capricious,  or  the  picture  of  gradually  advancing  stupidity, 
with  inability  to  grasp,  loss  of  initiative,  slowness  of  power  of  applica- 


'■  Franz,  Functions  of  the  Frontal  Lobes,  Archives  of  Psychology,  1907. 


502 


TUMORS  OF   THE  BRAIN 


tion,  is  seen.  In  right-handed  tumors,  the  psychical  disturbances  are 
more  frequent — Schuster^  (80  per  cent.),  PfeiflFer,^  Miiller.^ 

Orientation  for  the  external  world,  time  and  space,  is  apt  to  be 
involved  more  than  personal  orientation.  Complete  disorientation, 
as  in  Korsakow's  syndrome,  is  met  with. 

At  times  hallucinations  of  smell  appear  from  pressure  on  the  olfac- 
tory pathways,  or  hallucinations  of  sight,  photomata,  from  similar 
pressure  on  optical  pathways  at  the  base. 

Vertigo,  with  a  drunken  gait,  may  be  ascribed  probably  to  the  higher 
association  of  space  perceptions  and  indicates  frontal  involvement 
of  cerebellar  components,  the  gait  being  closely  related  to  that  of 
cerebellar  syndromes — the  patient  staggers  to  the  tumor  side:  adia- 
dokokinesia  and  asynergia  are  usually  absent  here,  however.  (See 
Cerebellar  Syndromes.) 


Fig.  245. — Frontal  lobe  tumor  removed.  This  patient  had  loss  of  smell  on  the 
tumor  side,  Witzelsucht,  staggering  gait,  and  loss  of  control  of  feces  at  times,  with 
otherwise  unimpaired  intelligence. 

Involuntary  defecation  or  urination  occurs  at  times;  most  frequently 
with  somnolent  patients.  The  patient  whose  tumor  is  here  figured 
had  a  marked  cerebellar  gait,  was  keen  and  active,  jocular  and 
happy,  with  occasional  loss  of  bladder  and  rectal  function  and  hallu- 
cinations of  smell.    The  tumor  occupied  the  left  frontal  pole  (Fig.  245). 

Apractic  disturbances  are  occasionally  met  with  in  frontal  tumors, 
and  those  involving  or  pressing  upon  Brocas'  convolution,  left  side, 
cause  mild  (paraphasic),  or  severe  motor  aphasias  in  right-handed 
individuals,  usually  of  gradual  onset  and  often  remittent  in  character. 

Other  motor  signs  are  stiffness  in  the  neck  with  forward  and  back- 
ward fixations  of  the  hand  and  tendencies  to  tremor  of  the  hand  on 


1  Thesis,  Stuttgart,  1902. 

2  Arch.  f.  Psych.,  xlvii. 

^  Deut.  Zeitschrift  f.  Nervenheilkunde,  vols,  xxi,  xxii,  xxiii. 


LOCAL  SYMPTOMS— CENTRAL  CONVOLUTIONS 


503 


the  tumor  side.     In  a  third  of  the  cases,  epileptiform  attacks,  often 
Jacksonian,  occur,  from  pressure  on  the  motor  area. 

Central  Convolutions. — ^The  functions  chiefly  involved  are  those 
of  the  voluntary  muscular  activity,  hence  paresis,  paralysis,  spasms. 
Tumors  of  this  region  are  never  latent.  Irritative  phenomena,  spasms, 
convulsions,  speak  for  cortical  locations;  paralysis  for  deeper-seated 
lesions  involving  the  pyramidal  paths  from  the  motor  areas.  Small 
tumors,  cortically  located,  cause  isolated  Jacksonian  attacks;  the 
more  extended  the  tumor  the  more  widespread  the  muscular  involve- 
ment; even  small  tumors,  however,  may  cause  widespread  Jacksonian 
or  grand  mal  symptoms.  Often  the  first  observed  motion  accompanied 
by  tingling  affords  a  clue  as  to  the  more  definite  localization  of  the 


Fig.  246. — Depression  in  brain  after  removal  of  a  frontal  tumor.     (Goodhart.) 


tumor;  again  an  orderly  and  uniform  progression  in  the  development 
of  a  Jacksonian  attack  is  valuable  in  localization. 

Monoplegias^  and  monopareses  are  not  infrequent  from  small  lesions, 
and  in  the  beginning  of  the  tumor  growth.  Th€  slow  extension  of  the 
paresis  or  paralysis  is  of  diagnostic  moment.  Advancing  hemiplegia 
is  often  accompanied  by  the  vasomotor  phenomena  seen  in  hemor- 
rhage— especially  in  deep-seated  lesions.  The  usual  signs  of  an  organic 
paralysis  {q.  v.)  are  present. 

Psychical  symptoms  of  general  nature  are  not  infrequent.  Occa- 
sionally large  tumors  will  cause  a  Korsakow  syndrome.     Katatonic 

1  Bergmark,  Monoplegia,  Brain,  1910. 


504 


TUMORS  OF   THE  BRAIN 


symptoms  may  also  appear.  Sensory  phenomena  are  frequent  in 
postcentral  convolution  tumors.  The  phenomena  have  been  exten- 
sively described  on  p.  479,  when  speaking  of  sensory  changes  due  to 
cortical  and  subcortical  lesions. 


Fig.  247. — Multiple  sarcoma  of  brain.     (Larkin.) 


Central  convolution  tumors  are  among  those  more  readily  localizable, 
and  usually  more  accessible  to  operative  relief. 

Parietal  Lohes. — When  the  tumors  press  forward  toward  the  pos- 
terior central  lobes  sensory  signs  are  produced  like  those  mentioned 
on  p.  479.  Left-sided  tumors,  in  right-handed  persons,  especially  of 
the  inferior  parietal  lobes,  cause  cortical  sensory  aphasia  of  Wernicke 


LOCAL  SYMPTOMS— TEMPORAL  LOBE  505 

of  various  grades.  Alexia  and  agraphia  may  also  be  foviiid  in  left-sided 
lesions. 

Epileptic  attacks  with  hallucinatory  auras  of  taste  occur;  also  a 
general  Korsakow  syndrome  may  develop.  Other  pathwaj^s  from  the 
sensor}^  areas  may  be  cut  off  by  tumors  in  this  region;  hence  either 
hemianopsias,  optic  agnosias  or  optical  aphasias  (gyrus  angularis). 
Apractic  disturbances  are  of  value  in  localizing  left-sided  tumors. 
Katatonic  syndromes,  confusion,  apathy,  or  general  loss  of  orientation 
may  be  encountered  but  are  equally  present  in  right-sided  and  left- 
sided  cases. 

Occasionally  parietal  tumors  give  rise  to  ptosis,  paresis  of  the 
lateral  movements  of  the  head  and  the  conjugate  motion  of  the  eyes 
to  the  opposite  side.  (Cephalorotary  and  oculorotary  paralysis,  see 
Plate  VIII.) 


^^^^^^^^^^^H^^TT 

^^¥^^^P^ 

W^^^V^ 

■■■ 

^              '^ 

■^- 

i% 

..'t_t.                       ■^  '3 

*""       Si' 

'  ^mS 

1^'^        "  nO^^^^^^^I 

mm 

Fig.  248. — Glioma  of  temporal  lobes. 

Deep-seated  lesions  may  impinge  upon  or  involve  the  motor  path- 
ways. 

Tem:iJoral  Lobes. — The  cortical  end  stations  of  the  auditor}^  path- 
ways which  are  both  crossed  and  uncrossed  are  contained  in  the 
first  and  second  temporal  lobes,  particularly  in  Heschl's  convolu- 
tions. Cortical  deafness  is  practically  impossible  in  unilateral  lesions 
but  has  resulted  from  bilateral  involvement. 

Word-deafness  is  the  most  striking  result  in  left-sided  lesions.  This 
is  a  progressive  affair,  often  beginning  with  difficulty  in  finding  words, 
paraphasia,  and  resulting  in  more  severe  forms  in  alexia,  agraphia, 


506 


TUMORS  OF  THE  BRAIN 


logorrhea,  and  total  word-deafness.  Large  tumors  also  cause  indirect 
symptoms  and  may  lead,  by  pressure  on  motor  areas— Broca — to 
total  aphasia,  and  epileptiform  convulsions. 

Auditory  hallucinations  are  not  infrequent,  showing  as  aurse  in 
generalized  grand  mal  attacks.  Gustatory  and  olfactory  phenomena 
of  similar  nature  result  from  hippocampal  or  closely  related  lesions — 
uncinate  fits. 

Tumors  of  the  under  surface  may  cause  hemianopsia,  through  press- 
ure on  the  optic  tracts,  and  by  pressure  on  the  pyramidal  or  fillet 
tracts  cause  hemiparesis  or  hemianesthesia. 

In  certain  cases,  as  Knapp^  has  shown,  there  may  be  an  orderly 
progression  of  symptoms  due  to  direct  or  indirect  action  in  temporal 
lobe  tumors.    The  most  striking  of  these  is  a  homo-  or  contralateral 


Fig.  249. — Tumor  of  temporo-occipital  lobes.     (Larkin.) 

temporary  or  persistent  oculomotor  palsy,  chiefly  ptosis  and  mydriasis, 
crossed  hemiplegia,  and  cerebellar  ataxia. 

Occipifal  Lobe. — The  end-projections  of  the  optic  tracts  are  located 
here  particularly  in  and  about  the  calcarine  fissure.  Complete 
homonymous  hemianopsia  is  the  chief  symptom  of  tumors  of  this 
area.  This  hemianopsia  usually  spares  the  papillomacular  bundles 
and  is  often  unperceived  by  the  patient.  Quadrant  hemianopsia  is  also 
found.  Tumors  may  exist  and  hemianopsia  be  absent.  It  is  most 
often  present  with  tumors  of  the  median  aspect  of  the  occipital,  also 
with  those  lying  on  the  convex  surface,  and  hence  the  more  readily 
removable.    Various  stages  of  blindness  may  also  result,  and  there. 

1  Die  Geschwiilste  d.  rechten  u.  linken  Schlafenlappens,  1905;  Miinch.  med.  Woch., 
1908. 


LOCAL  SYMPTOMS— CORPUS  CALLOSUM  507 

may  exist  a  mind-blindness  from  left-sided  tumors,  also  alexia,  agraphia 
and  sensory  aphasia. 

Other  optic  signs,  such  as  phonemes,  various  scintillating  scotomata, 
optical  hallucinations  and  illusions  occasionally  result  from  occipital 
tumors,  and  these  may  exist  as  auras  preceding  general  epileptic 
convulsions. 

Tumors  on  the  inferior  surface,  by  compression  of  the  cerebellum, 
will  cause  cerebellar  signs,  and  occasionally  palsies  result  from  tumors 
lying  on  the  external  surface  of  the  occipital  lobe. 


m 

w 

P 

% 

K 

"T! 

^ 

« 

■ 

\ 

9 

jg_,/^' 

^ 

^^M 

Y 

' 

1 

K,k*- 

V 

J 

1 

R 

H 

i 

9 

^k 

s 

/ 

^Hk; 

1 

t 

f 
i 

"^1 

Ml    jy* 

1 

r%''*  '*i 

1 

1 

k 

jj^M 

1 

1  ''*2 

mBM 

1 

1 

1 

1 

Fig.  250.— Tumor  arising  from  the  meninges  and  pressing  down  to  the  corpus  callosum. 

(Baldwin.) 

Corpus  Callosum. — Isolated  callosal  lesions  are  extremely  rare. 
Schuster  gathers  37;  Williams  38.^  Congenital  absence  is  known 
with  no  definite  symptoms.  Apraxia  (dyspraxia)  of  the  left  hand  is 
an  occasional  symptom  due  to  interruption  of  forward-lying  callosal 
fibers,  especially  those  between  the  sensorimotor  areas,  from  left  to 
right,  and  is  of  special  significance  in  diagnosis.  Possibly  mind-blind- 
ness results  from  lesions  of  those  callosal  fibers  uniting  the  two  optic 

1  Journal  of  Nervous  and  Mental  Disease,  1901. 


508  TUMORS  OF  THE  BRAIN 

fields  (Brodmann  18,  19),  also  termed  the  visual  psychic  area.    This 
is  not  yet  established  definitely.^ 

Paretic  syndromes  of  the  extremities  occur  with  callosal  tumors. 
Dupre  locates  them  in  the  anterior  callosal  region  if  the  paretic  symp- 
toms are  in  the  muscles  of  the  face  and  tongue;  pareses  and  ataxias 
of  the  upper  extremities  from  mid  callosal  fibers;  paresis,  ataxia  and 
hemianopsia  from  posterior  callosal  fibers.  Van  Valkenberg's  exten- 
sive studies  of  the  callosum  do  not  at  all  corroborate  Dupre's 
deductions. 


Fig.  251. — Tumor  with  atrophy  of  the  cerebellum. 

Tumors  of  the  callosum  are  more  apt  to  show  compound  symptoms 
due  to  involvement  of  the  neighboring  parts;  if  lying  forward,  frontal 
signs  are  added;  midcallosal  region,  pyramidal  tract,  epilepsies,  etc. 
General  psychical  symptoms  are  usually  present,  and  appear  early. 
They  approach  the  type  due  to  general  pressure.  Korsakow  and 
paretic  syndromes  develop.  There  is  nothing  diagnostic  about  the 
mental  signs,  save  as  might  appear  from  the  neighboring  pressed  upon 
areas. 

Tumors  of  the  Optic  Thalamus. — ^The  symptoms  are  discussed  under 
the  section  on  the  thalamic  syndrome  (g.  v.).     Involvement  of  the 

1  Van  Valkenberg,  Brain,  November,  1913. 


TREATMENT  509 

lenticular  nucleus  (see  Wilson's  disease),  tumors  of  the  midbrain, 
pons  and  medulla  have  been  discussed.  Those  of  the  cerebellum  and 
cerebellopontine  angle  will  be  found  under  Cerebellar  Syndromes. 

Diagnosis. — INIultiple  sclerosis,  paresis,  arteriosclerotic  disease,  all 
forms  of  headache,  tuberculous  meningitis,  chronic  hydrocephalus, 
and  hysteria  are  the  chief  conditions  causing  difficulty. 

Multiple  sclerosis,  if  the  patches  are  solely  cerebral,  may  cause 
confusion,  especially  in  the  acute  cases,  as  described  by  Marburg  and 
others.  The  bitemporal  pallor  of  the  disks  in  this  disorder  differs 
from  the  usual  pressure  changes  in  the  disk.  Nystagmus  is  not  a 
frequent  brain-tumor  sign.  Other  signs  of  pyramidal  tract  implica- 
tion may  be  identical.  Headaches  are  usually  absent,  also  nausea  and 
vomiting. 

Pseudoparetic  and  arteriosclerotic  psychical  syndromes  (Korsakow's 
psychosis)  are  frequently  confused  with  cerebral  tumor,  i.  e.,  the  cause 
of  the  mental  picture  is  overlooked.  Thus  a  tumor,  which  might  have 
been  removed,  has  been  missed  under  the  psychotic  disguise.  Eye- 
ground  changes  are  usually  positive  in  these  cases,  yet  may  be  absent. 

Headache  should  always  be  scrutinized  carefully.  A  postinflu- 
enzal occipital  headache  which  is  very  frequent  and  extremely 
severe  and  persistent,  is  frequently  highly  suggestive  of  brain  tumor. 
The  headaches  from  lead  poisoning,  anemia,  and  nephritis  are  also  to 
be  excluded. 

Brain  abscess  and  tuberculous  meningitis  must  be  excluded  on  the 
ground  of  their  difference  in  development  of  symptoms.  Symptomati- 
cally  speaking  they  may  be  considered  as  tumors.  Hence,  see  Symp- 
toms, p.  462.    This  is  also  true  of  chronic  hydrocephalus. 

Cerebral  puncture,  withdrawing  a  small  plug  of  brain  tissue  through 
a  trephine  opening  and  canula  is  often  of  great  aid  in  diagnosing  very 
puzzling  cases. 

Prognosis. — No  definite  prognosis  can  be  laid  down.  Everything 
depends  on  the  site  of  the  tumor.  In  general,  apart  from  surgical  relief 
and  from  medicinal  treatment  of  syphilomata,  the  outlook  is  pessi- 
mistic. Sudden  death  is  not  infrequent  and  lumbar  puncture  is  an 
extremely  dangerous  procedure  with  brain  tumor,  often  leading  to 
sudden  collapse  and  death,  especially  with  tumors  of  the  posterior 
fossa. 

Syphilomata  and  gummata  of  the  brain  have  a  fair  prognosis.  Better 
results  are  obtained  with  mercury  by  inunction  and  by  iodides  than 
by  salvarsan  in  the  beginning  treatment.  Salvarsan  may  be  used 
later  to  attempt  to  kill  off  all  the  spirochetes,  but  with  well-advanced 
syphilomata,  salvarsan  is  apt  to  set  up  a  dangerous  reaction.  Round, 
hard  gummata  do  not  absorb,  as  a  rule,  and  are  best  considered 
surgically. 

Treatment. — Medicinal  treatment,  excepting  for  syphilomata,  is 
useless,  and  involves  a  waste  of  valuable  time.  The  chief  objects  to  be 
attained  are  early  diagnosis,  immediate  exclusion  of  syphilis  by  sero- 


510  TUMORS  OF   THE  BRAIN 

logical  tests,  eye-ground  examination  with  particular  study  of  the 
color  fields,  exact  localization  as  soon  as  possible,  and  surgical  removal 
or  palliation  (decompression)  to  save  the  eyesight  or  to  gain  time  for 
a  more  exact  localization. 

The  details  for  applying  these  principles  have  already  been  noted. 
The  results  to  be  expected  in  any  particular  case  are  problematical, 
yet  from  10  to  20  per  cent,  of  all  brain  tumors  (seen  in  the  large) 
have  been  removable,  with  at  least  in  10  per  cent,  practical  recovery. 
Even  with  such  chances  against  him  the  patient  should  have  the 
benefit  of  the  doubt  if  a  competent  surgeon  is  available.  Surgical 
skill  is  a  very  large  factor  in  the  results;  a  good  abdominal  surgeon 
is  not  necessarily  a  good  brain  surgeon.  The  brain  is  semifluid  and  an 
intricate  switch-board  of  highly  important  structures;  there  are  no 
unimportant  areas  in  the  brain;  many  surgeons  have  treated  it  in  the 
past  as  though  it  were  an  abdominal  viscus.  The  results  have  been 
disappointing. 

Most  brain  operations  are  best  done  in  two  stages.  Decompression, 
usually  subtemporal,  alone  is  often  the  only  possible  procedure.  It 
often  relieves  a  recently  acquired  blindness. 

The  situation  referable  to  brain  surgery  for  cerebral  tumors  is 
rapidly  advancing,  and  better  and  better  results  are  being  obtained 
and  regions  hitherto  impossible  to  reach  (hypophysis,  etc.)  have  been 
approached  with  results  which  a  decade  ago  would  have  been  impos- 
sible.^ Notwithstanding  all  this,  the  general  attitude  should  be  one 
of  extreme  caution. 

1  See  Starr,  Brain  Surgery;  Halsted,  Treatment  of  Brain  Tumors;  White  and  Jelliffe, 
Modern  Treatment  of  Nervous  and  Mental  Disease. 


CHAPTER  XV. 
SYPHILIS  OF  THE  NERVOUS  SYSTEM. 

Syphilis  of  the  nervous  system  is  so  protean  in  its  clinical  forms, 
that  the  significance  of  syphilis  as  an  etiological  factor  in  nervous 
diseases  frequently  is  entirely  overlooked.  The  physician  with  his  eye 
riveted  upon  a  clinical  picture,  be  it  an  amyotrophic  lateral  sclerosis, 
a  failing  memory,  a  persistent  nervous  weakness,  a  progressive 
muscular  atrophy,  or  a  protracted  sciatica,  and  mindful  of  the  somber 
prognosis  given  in  the  majority  of  his  available  guides,  may  readily 
overlook  the  fact  that  syphilis  is  the  cause  for  these  syndromes. 

There  is  no  field  in  medicine  wherein  similar  diseased  pictures  may 
arise  from  as  many  differing  causes  as  in  the  domain  of  the  nervous 
system.  Nor,  on  the  other  hand,  where  a  single  etiological  factor  may 
give  rise  to  so  many  dissimilar  clinical  pictures.  Hence,  the  com- 
plexity of  the  whole  subject,  and  the  need  for  iterating  and  reiterating 
the  advice  that  in  nearly  all  of  the  clinical  pictures  which  have  or  have 
not  been  given  descriptive  terms  in  nervous  or  mental  disease,  the 
possibility  of  syphilis  as  a  direct  etiological  factor  or  as  a  complica- 
tion, or  as  causing  confusion,  should  be  borne  in  mind.  Hence,  the 
necessity  for  detailed  and  minute  inquiries  into  all  of  the  possible 
symptomatology,  of  hereditary  or  acquired  syphilis,  which  in  all 
questionable  cases  should  be  supplemented  by  complete  serological 
and  cytological  examinations. 

The  formerly  very  distinct  dividing  lines  between  the  lesions  of 
hereditary  syphilis,  acquired  syphilis,  and  metasyphilis  are  slowly 
being  obliterated,  and  in  time  it  may  probably  seem  strange  that  it 
could  have  been  thought  that  spinal  and  cerebral  syphilis  on  the  one 
hand  should  ever  have  been  considered  different,  let  us  say,  from  tabes 
and  general  paresis  on  the  other. 

Since  it  has  come  to  be  believed  that  both  tabes  and  general  paresis 
rest  upon  a  syphilitic  basis,  the  variety  of  syphilitic  disorders  showing 
fairly  clear  clinical  entities  has  been  enlarged.  Further,  with  the 
recognition  of  many  acute  and  subacute  psychoses  due  to  syphilis 
the  psychiatric  borders  have  been  further  extended. 

In  the  present  chapter,  then,  the  discussion  will  concern  itself  chiefly 
with  clinical  forms,  without  any  extended  attempt  being  made  to 
differentiate  types,  which  in  reality  are  so  kaleidoscopic  that  they 
defy  description. 

At  the  same  time  it  needs  to  be  emphasized  that  such  intermediary 
forms  are  ever-present  realities.     Classical  pictures  of  a  disease  are 


512  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

largely  literary  efforts.  They  are  the  product  mainly  of  the  descrip- 
tive art.  The  actual  processes  going  on  in  nature  in  the  conflict  of 
man  against  the  spirochete,  does  not  show  classical  types  but  a  multi- 
plicity of  variations,  with  here  and  there  statistical  prominence  of 
this  or  that  trend  in  the  reactive  compromise. 

History. — Leoncino  as  early  as  1497  described  paralysis  as  a  con- 
sequence of  the  disease.  He  here  referred  to  what  is  known  as  hemi- 
plegia, which  may  result,  as  is  well  known,  within  a  few  years,  even 
a  few  months,  after  infection. 

Joseph  Griinbeck  (1503),  Emser  (1511),  Ulrich  von  Hutten  (1519), 
all  lay  writers,  mention  paralyses  of  the  limbs  as  due  to  the  disease. 
Emser  speaks  of  his  patient,  a  syphilitic  paralytic,  and  with  a  psychosis, 
as  having  made  a  remarkable  recovery,  under  treatment  by  Bruno, 
by  making  a  vow.  Paracelsus  (1530),  although  still  confusing  gon- 
orrhea with  syphilis,  as  had  been  and  is  still  being  done,  left  indica- 
tions of  a  description  of  syphilitic  meningitis,  and  in  speaking  of  the 
syphilitic  virus  said  that  it  affected  all  of  the  organs  of  the  body. 
Nowhere  in  Fracastorius  (1521),  who  gave  the  name  syphilis,  are  direct 
references  to  the  nervous  system  to  be  found.  Nicolaus  Massa  (1556) 
gave  an  early  description  of  syphilitic  neuralgias.  Borgarutius  (1567) 
also  described  neuralgic  pains  due  to  syphilitic  disease  of  the  meninges. 
Amatus  Lusitanus  (1561)  described  headaches  due  to  intracranial 
osteitis  of  syphilitic  origin.  Botalli  (1563)  made  an  observation  that 
blindness  might  be  due  to  syphilitic  disease  of  the  brain. 

During  the  following  century  many  references  have  been  found 
showing  the  recognition  of  the  relationship  of  syphilis  to  nervous 
disease.  Only  a  few  can  be  mentioned  here.  Thus,  Guarinoni  (1610) 
described  epileptic  attacks  from  syphilis  of  the  brain.  Vidus  Vidius 
(1611)  described  epilepsies  as  due  to  syphilitic  cranial  caries.  Thiery^ 
de  Hery  (1634)  and  Zechius  (1650)  also  called  attention  to  syphilitic 
spasms  as  well  as  epilepsies.  Zacutus  Lusitanus  (1644)  described 
cases  of  blindness  due  to  gumma  of  the  brain,  quoting  Botalli  a  century 
ahead  of  him.  In  1696  a  special  treatise  on  syphilitic  pains  was  written 
by  Blagny. 

Attention  might  be  called  to  the  works  of  Scholtzius  (1610)  and 
Willis  (1672)  a  propos  of  the  subject  of  general  paresis.  To  Willis 
has  always  been  ascribed  the  honor  of  the  first  description  in  which 
one  could  definitely  recognize  general  paresis. 

By  the  end  of  this  (seventeenth)  century  a  fairly  broad  view  of 
syphilitic  nervous  disease  had  been  obtained.  Syphilitic  headaches 
were  described  by  Felix  Plater  (1641).  Rhodius  (1657)  described 
gummata  of  the  dura  and  syphilitic  hemiplegias;  Ballen  (1663),  spinal 
syphilitic  disease  and  spasms  in  the  facial  region;  Cummius  (1684), 
diplopias  and  eye  palsies.  Astruc  has  reviewed  these  writings 
completely. 

During  the  eighteenth  century  the  picture  expanded  rapidly. 
Intercostal  neuralgias  (1762),  deafness,  loss  of  smell,  caries  of  base  of 


HISTORY  513 

skull  (1762),  sciatica  (1745),  psychoses.  Syphilitic  mania  was  described 
by  Sanche  in  1777,  amaurosis  (1748),  facial  palsy  (1758),  leptomenin- 
gitis (1766),  syphilitic  arterial  disease  (1766),  paraplegias  (myelitis) 
(1771),  and  a  number  of  other  conditions  were  described,  and  may 
be  consulted  in  Lagneau's  interesting  monograph  in  which  234  case 
histories  are  collected.  Astruc,  Bonet,  and  Morgagni  offer  the  richest 
literary  sources. 

It  may  be  recalled  in  this  place  that  John  Hunter  in  1787  stated 
that  he  never  observed  syphilis  in  the  internal  organs,  including  the 
brain.  The  weight  of  his  authority  retarded  progress  for  many  years, 
especially  in  England.  Indeed,  it  was  not  until  Ricord's  sound 
observations  were  published  that  Hunter's  enormous  blunder  was 
fully  remedied. 

Virchow's  studies  (1847)  on  phlebitis  and  arteritis  had  laid  the 
foundation  for  our  modern  knowledge  of  bloodvessel  syphilis,  although 
it  may  be  recalled  that  Morgagni  (1766)  and  Home  (1782)  both  made 
extremely  important  studies  on  vascular  syphilis.  These  have  been 
fully  developed  by  Huebner  (1874)  and  Alzheimer  (1904). 

The  studies  of  Virchow  on  the  formation  of  gummatous  granulo- 
mata  and  related  syphilitic  phenomena,  practically  established  the 
modern  era  of  study  of  the  pathology  of  this  disease. 

The  succeeding  years  have  filled  in  the  picture  with  a  number  of 
details,  the  chief  additions  having  been  those  of  Nissl  and  Alzheimer, 
who  have  established  the  highest  criteria  for  the  pathology  of  this 
disease  so  far  as  the  nervous  system  is  concerned. 

The  latest  chapter  in  this  interesting  history  is  that  dealing  with 
the  discovery  of  the  exciting  agent,  and  the  final  clearing  up  of  the 
entire  subject  of  etiology  and  modes  of  infection.  Schaudinn  (1905) 
demonstrated  the  parasite  which  he  called  Spirochaeta  pallida.  Its 
varied  synonymy  to  accord  with  principles  of  botanical  and  zoological 
nomenclature  need  not  detain  us.  Spironema  pallida,  Treponema 
pallidum  are  those  most  frequently  employed.  Doele  (1892)  is  thought 
to  have  first  seen  the  parasite,  but  Schaudinn,  then  Epaschen,  Fischer, 
Metchnikoff,  and  Roux  established  its  identity  and  its  affiliations. 

It  is  found  in  congenital  syphilis  of  the  nervous  system,  in  syphilitic 
gumma  of  the  brain  and  spinal  cord,  in  syphilitic  meningitis,  even  in 
the  cerebrospinal  fluid,  both  of  congenital  and  acquired  syphilitics. 
Moore  (1913),  Noguchi,  Nichols,  and  Hough  found  it  in  the  paretic 
brain,  and  it  has  been  reported  to  have  been  found  in  the  spinal 
meninges  of  tabetics. 

The  final  studies  of  Neisser,  Metchnikoff,  Roux,  and  others  have 
laid  bare  the  entire  story  of  the  inoculability  of  the  disease  and  its 
transmission  from  animal  to  animal,  while  utilizing  the  knowledge 
gained  by  Bordet  and  Gengou,  Wassermann  and  his  pupils  have 
elaborated  a  serobiological  technique  which  has  made  one  independent 
of  clinical  or  anamnestic  data  relative  to  a  knowledge  on  the  part  of 
the  patient  of  the  infection. 
33 


514  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

Thus,  in  hardly  more  than  seven  years,  a  flood  of  Hght  has  been 
thrown  upon  the  disease  and  its  relation  to  other  protozoal-caused 
diseases,  notably  trypanosomiasis,  which  has  illuminated  and  made 
clear  the  entire  path  which  has  been  so  busily  travelled  since  Columbus 
brought  back  this  most  portentious  exotic  to  the  old  world. 

Diagnosis. — The  diagnosis  of  syphilis  of  the  nervous  system  presents 
certain  difficulties  which  rapidly  increasing  perfections  in  laboratory 
technique  are  resolving  with  considerable  success.  These  laboratory 
findings,  combined  with  those  of  the  neurological  examination,  permit 
an  almost  certain  diagnosis  of  this  disease  in  the  nervous  system,  either 
as  congenital,  acquired,  or  as  para-  or  metasyphilis. 

The  chief  features  in  such  diagnosis  are:  (1)  search  for  the  organ- 
isms; (2)  serological  investigation  of  the  blood  and  cerebrospinal 
fluid;  (3)  cytological  examination  of  the  cerebrospinal  fluid;  (4) 
chemical  examination  of  the  cerebrospinal  fluid;  (5)  clinical  examina- 
tion of  the  pupillary  reflexes. 

1.  Search  for  Organisms. — The  parasite  has  been  found  in  the 
cerebrospinal  fluid,  but  as  yet  in  but  few  instances.  It  has  been 
cultivated  from  the  cerebrospinal  fluid  (Nichols  and  Hough) .  Increas- 
ing experience  along  these  paths  of  study  will  probably  develop  impor- 
tant diagnostic  aids.  These  are,  as  yet,  of  less  practical  use  than 
others  to  be  enumerated. 

2.  Biological  or  Serological  Tests  of  Blood  and  Cerebrospinal  Fluid. — ■ 
(a)  Blood. — Practically  all  forms  of  early  syphilis  of  the  nervous 
system  should  show  a  positive  Wassermann  reaction  in  the  blood. 
There  are  exceptions,  but  syphilis  of  the  nervous  system,  like  syphilis 
elsewhere,  gives  a  positive  reaction  in  early  cases. 

Whereas,  the  number  of  positive  findings  should  be  as  high  as 
100  per  cent,  in  cases  of  early  syphilis  of  the  nervous  system,  in  its 
secondary  and  tertiary  stages  the  number  of  positive  results  may 
fall  to  even  70  per  cent.,  and  in  the  latent  period  may  sink  to  50  per 
cent.  Whether  these  reduced  percentages  speak  for  the  good  results 
of  treatment  or  are  dependent  upon  other  as  yet  uncertain  factors 
is  yet  to  be  decided.  The  alcohols  interfere  with  the  reaction.  Hence 
the  results  of  a  Wassermann  test  are  apt  to  be  unreliable  if  the  patient 
has  taken  alcohol  within  forty-eight  hours  of  the  time  of  making  the 
test. 

(6)  Cerebrospinal  Fluid.— The  behavior  of  the  cerebrospinal  fluid 
to  the  Wassermann  reaction  is  of  special  significance  in  the  diagnosis 
and  treatment  of  syphilis  of  the  nervous  system.  It  is  almost  uniformly 
positive  in  general  paresis,  even  when  small  quantities  (0.02  c.c.)  of 
the  serum  are  employed.  By  the  use  of  such  small  quantities  it  would 
appear,  from  the  work  of  Hauptmann  and  Hossli,  that  paresis  alone 
will  cause  a  positive  result,  but  with  larger  quantities  of  cerebrospinal 
fluid  (0.4  to  0.8  c.c),  practically  all  forms  of  cerebrospinal  syphilis  will 
give  a  positive  reaction;  tabes,  cerebral  syphilis,  meningomyelitis,  etc. 
(Hauptmann,  Holtzmann,  Swift  and  Ellis.) 


DI A  GNOSIS— CEREBROSPI NAL  FLUID 


515 


Syphilis  without  nervous  involvement  usually  gives  a  negative 
reaction,  even  when  large  quantities  of  the  fluid  are  used. 

It  must  constantly  be  borne  in  mind  in  the  diagnosis  of  syphilis  of 
the  nervous  system  that  the  reaction  of  the  cerebrospinal  fluid  in  the 
Wassermann  test  is  purely  monosymptomatic.  The  positive  or  nega- 
tive results  must  always  be  interpreted  by  association  with  other 
laboratory  and  clinical  tests.  As  Nonne  has  well  said,  the  Wasser- 
mann reaction  is  only  a  symptom.  Like  other  symptoms  in  a  syndrome 
it  may  or  may  not  be  present  without  affecting  the  validity  of  the 
syndrome  from  its  diagnostic  aspects. 


Fig.  252. — Lymphocytes  in  normal  (left)  and  paretic  (right)  cerebrospinal  fluid, 

(Kraepelin.) 


3.  Cytological  Examination  of  the  Cerebrospinal  Fluid. — The  tech- 
nique of  puncture  cannot  be  entered  into  here,  but  one  point  should 
be  borne  in  mind:  the  procedure  is  not  always  without  danger.  It 
should  be  done  with  care,  the  fluid  being  withdrawn  very  slowly,  drop- 
wise  in  some  cases,  and  the  patient  should  rest  in  bed  several  hours, 
preferably  both  before  and  after  the  operation.  One  of  the  functions 
of  the  cerebrospinal  fluid  is  to  maintain  an  equality  in  the  intracerebral 
pressure,  and  any  sudden  alteration,  such  as  is  produced  by  the  with- 
drawal of  5,  10,  or  15  c.c.  of  the  fluid,  is  apt  to  disturb  such  equilibrium. 
Headache,  nausea,  vomiting,  dizziness,  are  among  the  unpleasant 
effects  in  those  who  react  badly.  Such  are  few,  but  they  exist,  and 
care  is  imperative.    Some  are  helped  by  lying  quiet,  with  foot  of  bed 


516  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

elevated  and  with  aspirin,  10  grains,  repeated  if  necessary.  In  patients 
in  whom  brain  tumor  is  suspected  special  care  should  be  taken,  as 
here  a  few  cases  of  sudden  death  have  been  reported  following  lumbar 
puncture  with  withdrawal  of  fluid. 

In  pathological  cerebrospinal  fluid  an  increase  in  lymphocytes  is 
the  most  striking  feature.  Over  7  to  10  lymphocytes  to  the  c.mm. 
indicates  pathological  fluid.  Other  cells  may  also  be  found,  such  as 
polymorphonuclear  leukocytes,  plasma  cells,  and  occasionally  eosino- 
phile  and  endothelial  cells.  Red  blood  cells  usually  come  from  the 
wound  of  the  puncture. 

The  fluid  is  best  fixed  and  imbedded,  and  then  stained  by  the 
Alzheimer  method.  This  is  the  most  complete  and  satisfactory 
method  thus  far  devised,  as  it  permits  of  a  counting  and  study  of  all 
the  cells.  The  Fuchs-Rosenthal  counting  chamber,  however,  is  that 
used  in  the  greater  number  of  instances.  Its  results  are  more  quickly 
obtained,  although  they  lack  the  finality  of  the  Alzheimer  method. 

A  normal  cerebrospinal  fluid  is  a  clear  liquid.  It  has  a  specific 
gravity  of  1006,  a  slightly  alkaline  reaction,  and  is  almost  free  from 
cellular  constituents — 1  to  5  lymphocytes  per  c.mm.,  as  estimated  by 
the  Fuchs-Rosenthal  method,  may  be  considered  normal.^ 

Changes  in  Cerebrospinal  Fluid. — In  cerebrospinal  syphilis,  in  tabes, 
and  in  general  paresis  there  is  usually  an  increase  in  the  number  of 
lymphocytes.  They  may  run  up  into  the  hundreds,  especially  in 
some  cases  of  acute  meningomyelitis;  also  in  some  fulminating  cases 
of  paresis.  In  fact,  most  meningitic  processes  are  accompanied  by 
increase  in  cellular  content.  Polynuclear  cells  are  frequent  in  the 
acute  types,  sometimes  tuberculous  meningitis  excepted,  and  as  the 
process  tends  to  chronicity  there  is  a  tendency  to  lymphocytosis  and 
loss  of  other  cell  types.  The  pleocytosis  of  syphilis  has  usually  been 
attributed  to  a  meningitic  process.  It  has  been  suggested  as  due  to  a 
periarteritis  as  well  (Szecsi). 

Certain  general  variations  may  be  recorded.  The  earlier  students 
were  more  didactic  in  their  statements  concerning  specific  differences 
in  the  number  of  cells  as  distinguishing  cerebrospinal  syphilis,  tabes, 
and  paresis.  Further  extension  of  the  studies  shows  them  to  have 
been  in  part  unwarranted.  Thus  it  has  been  said  that  the  lower 
numbers  point  to  cerebrospinal  syphilis,  the  higher  number  to  tabes, 
and  the  highest  to  paresis.  This  is  perhaps  so,  but  it  is  not  an  absolute 
rule.  The  number  of  cells  seems  to  bear  a  more  definite  relation  to 
the  activity  of  the  underlying  irritation  or  inflammatory  process  than 
to  its  kind.  Thus  a  stationary  tabes  may  show  few  cells,  also  a  paresis 
in  remission,  whereas  an  acute  cerebrospinal  syphilis  or  an  acute  menin- 
gomyelitis may  show  many  cells. 

A  fluid  rich  in  polymorphous  cells  is  indicative  of  a  very  active 
process,  syphilitic  or  otherwise. 

1  See  Thomsen,  Hill,  Halliburton,  Proc.  Roy.  Soc,  vol.  Ixiv,  for  normal  fluid. 


DIAGNOSIS— CEREBROSPINAL  FLUID  517 

In  cerebrospinal  syphilis,  tabes,  and  particularly  in  paresis,  it  is  of 
great  importance  to  note  that  a  pleocytosis,  oftentimes  of  very  marked 
grade,  may  antedate  all  neurological  symptoms  of  the  after-coming 
disorder.  This  has  been  shown  repeatedly  by  Sicard,  by  Ravaut  and 
others.  This  preparetic  period  has  been  diagnosticated  as  long  as 
two  years  before  the  onset  of  the  disease.  This  point  is  well  to  bear 
in  mind  when  one's  advice  is  asked  as  to  the  advisability  of  marriage 
of  syphilitics  in  the  forties.  It  is  not  at  all  an  infrequent  experience 
to  find  the  outbreak  of  paresis  occurring  in  men  of  from  forty  to 
forty-five,  who  have  been  from  one  to  three  years  married,  and  many 
have  waited  this  long,  feeling  that  because  of  an  early  syphilis  it  were 
wise  to  defer  marriage  until  a  safe  period.  A  return  will  be  made  to 
the  prophylactic  features  later  in  the  more  detailed  discussion  of  the 
therapy. 

How  long  after  infection  by  syphilis  may  lymphocytosis  appear  in 
the  cerebrospinal  fluid?  Varying  answers  are  available,  but  Ravaut 
has  reported  its  presence  at  least  two  months  after  infection.  The 
presence  of  lymphocytes  almost  invariably  speaks  for  nervous  syphilis. 
In  patients  who  have  thus  far  been  followed,  the  development  of 
nervous  accidents,  hemiplegia,  paraplegia,  meningomyelitis,  etc.,  has 
taken  place. 

With  active  therapy  the  pleocytosis  is  apt  to  disappear.  Are  those 
patients  who  retain  their  lymphocytosis  on  the  way  to  tabes  or 
paresis?  This  is  an  important  problem  which  has  been  answered 
yes  and  no.  Its  definite  answer  is  yet  to  come,  as  the  necessary  time 
during  which  the  full  significance  of  the  results  of  cytological  study  of 
the  fluid  has  not  yet  elapsed. 

From  the  therapeutic  point  of  view  then  the  whole  subject  of  pleo- 
cytosis of  the  cerebrospinal  fluid  is  full  of  significance,  and  in  need 
of  constant  attention.  In  this  connection  it  should  again  be  empha- 
sized that  lymphocytosis  alone  does  not  mean  syphilis  alone.  It  can 
be  said  that  absence  of  lymphocytes  at  least  negatives  tabes  and 
paresis — to  this  generalization  there  are  but  few  recorded  negative 
observations.^ 

Lymphocytosis  is  not  limited  to  syphilis  of  the  nervous  system, 
however.  It  is  marked  in  sleeping  sickness,  an  allied  disease  (Spiel- 
meyer,  Mott).  It  is  often  also  high  in  tuberculous  meningitis,  but 
is  here  usually  complicated  by  the  presence  of  polymorphonuclear 
leukocytes.  In  a  few  cases  of  multiple  sclerosis  lymphocytes  have 
been  found.  In  the  acute  stages  of  poliomyelitis  lymphocytes  may 
be  found,  also  in  epidemic  cerebrospinal  meningitis  and  in  herpes 
zoster. 

4.  Chemical  Examinatioii. — Here  the  presence  of  a  reducing  agent 
(Fehling's)  and  of  globulin  is  to  be  estimated.     Most  fluids  contain 

1  Klieneberger,  Archiv  f.  Psychiatrie,  1911;  Foster,  Lewandowsky,  Handbuch  der 
Neurologic. 


518  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

the  former.    When  present  in  large  quantities  it  may  point  to  a  tuber- 
culous meningitic  process. 

Increased  globulin  content  is  a  characteristic  feature  of  paresis. 
In  tabes  increased  globulin  is  the  rule^  as  is  also  the  case  in  cerebro- 
spinal syphilis,  but  to  a  less  extent.  Markedly  increased  globulin 
content  is  not  infrequent  in  spinal  cord  tumors,  gummatous  or  non- 
syphilitic.  The  globulin  reaction  is  apt  to  run  along  with  the  lympho- 
cytosis. It  has  no  apparent  alliances  with  the  findings  of  the 
Wassermann  test. 

Summary  of  Laboratory  Findings. — Four  Reactions. — Before  passing 
to  the  diagnostic  significance  of  the  clinical  examination  of  the  eye 
reflexes  a  word  should  be  said  relative  to  the  value  of  these  "four 
reactions,"  as  Nonne  has  called  them.  It  has  been  said  that  taken 
alone  they  may  mean  nothing  positive,  so  far  as  a  differential  of  the 
different  types  of  syphilis  of  the  nervous  system  is  concerned,  but 
when  read  together  they  afford  important  guides  to  diagnosis  and  to 
treatment. 

Nonne 's  conclusions  are  perhaps  the  most  extensive  that  we  possess 
on  this  point.  They  are  founded  upon  his  own  experience  with  167 
patients  with  tabes,  179  with  paresis,  97  with  cerebrospinal  syphilis 
(arterial  types),  68  patients  with  multiple  sclerosis,  38  with  brain 
tumor,  and  14  with  spinal  cord  tumor. 

Expressed  schematically,  which  schemes,  as  he  well  remarks,  are 
not  products  of  nature  but  of  man,  the  following  results  of  the  four 
reactions  are  to  be  expected:     (I)  blood  and  (II)  fluid. 
I.  Blood  examination. 
Wassermann  reaction. 

(a)  Positive.    Is  characteristic  of  syphilis  with  few  exceptions 

(already  noted).  A  positive  Wassermann  of  the  blood 
serum  says  nothing  further  than  that  the  individual  has 
come  in  some  manner  in  contact  with  syphilis,  either 
through  heredity  or  by  infection.  It  does  not  say  that 
the  disease  from  which  he  suffers  is  due  to  syphilis. 

(b)  Negative.      Is   dift'erentially   diagnostic   against   paresis, 

since  it  is  only  very  rarely  that  the  blood  in  paresis  gives 

a  negative  reaction. 
II.  Study  of  cerebrospinal  fluid. 

(a)  Normal  fluid.    Pressure  90  to  130  mm.  water.    Globulin 

reaction   negative — not   over   5   or   6   cells   to   c.mm. 

(Fuchs-Rosenthal) . 
(6)  Pathological  fluids. 

1,  Increased  pressure — over  15  c.mm.  water. 

2.  Positive  phase  I.    Globulin  reaction. 

3  Increased  cell  count.  (These  three  symptoms,  in 
coordination  or  alone  indicate  the  presence  of  an 
organic  nervous  disorder,  syphilitic  or  non- 
syphilitic.) 


DIAGNOSIS— CEREBROSPINAL  FLUID  519 

(c)  If  the  disease  of  the  nervous  system  is  syphiUtic,  then  the 
Wassermann  test  of  the  fluid  will  show.  If  the  Wasser- 
mann  reaction  (original  method — 0.2  c.c.  of  the  fluid)  is 
positive,  there  is  great  probability  that  the  patient  is  a 
paretic,  or  a  taboparetic,  much  less  often  a  cerebro- 
spinal syphilitic,  or  a  pure  tabetic.  In  nearly  all  cases 
of  cerebrospinal  syphilis  and  of  tabes  the  Wassermann 
reaction  becomes  positive  by  using  0.4  to  1  c.c.  of  fluid. 
Nonne's  typical  findings  are  as  follows: 
I.  Paresis  or  taboparesis. 

1.  Wassermann  reaction  in  blood  positive  (100  per  cent.). 

Pressure  increased. 

2.  Phase  I,  globulin  reaction  positive  (95  to  100  per  cent.). 

3.  Lymphocytosis  (95  per  cent.). 

4.  Wassermann  in  fluid. 

(a)  Positive— about  85  to  90  per  cent,  with  original 

method  and  0.2  c.c.  fluid. 
(h)  Positive  in  100  per  cent,  with  larger  quantities  of 

fluid. 
II.  Tabes  without  paresis. 

1.  Wassermann  reaction  in  blood  positive  (60  to  70  per  cent.). 

Pressure  usually  increased. 

2.  Phase  I,  reaction  globulin  and  positive  (90  per  cent.). 

3.  Lymphocytosis  positive  (90  per  cent.). 

4.  Wassermann  in  fluid. 

(a)  Original  method,  0.2  c.c.  positive  (5  to  10  per  cent.). 
(6)  Larger  quantities  (100  per  cent.). 
III.  Cerebrospinal  syphilis. 

1.  Wassermann  reaction  in  blood  positive  (80  to  90  per  cent.). 
Pressure  frequently  increased. 

2.  Phase  I,  reaction  usually  positive,  exceptionally  negative. 

3.  Lymphocytosis  nearly  always  positive. 

4.  Wassermann  in  fluid. 

(a)  Original  methods  (0.2  c.c.)  positive  in  about  10  per 

cent. 
(6)  Larger  quantities  nearly  always  positive  (of  value 
in  diagnosis  of  multiple  sclerosis,  cerebral  and 
spinal  tumor). 
These  results  of  Nonne's  summarize  fairly  accurately  the  present-day 
attitude  on  the  value  of  the  four  reactions.     The  full  significance 
of  the  findings  can  be  gained  only  by  reference  to  the  originals.    This 
field  of  work  is  rapidly  expanding,  and  that  which  now  appears  on  the 
frontiers  of  our  knowledge  will  undoubtedly  be  much  modified  by  the 
rapidly  advancing  army  of  investigators. 

Additional  Tests. — These  additional  tests  are  valuable  because 
syphilis  may  be  present  with  a  negative  Wassermann.  These  cases 
of  so-called  latent  syphilis  with  negative  Wassermann  occur  in  about 


520  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

35  per  cent,  of  cases  with  no  active  signs  of  the  disease  but  with  a  clear 
history  of  infection. 

The  Liietin  Test. — This  is  a  cutaneous  test  with  a  suspension  of 
killed  imllida  cultiu-es  as  prepared  by  Noguchi,  known  as  luetin.  A 
positive  reaction  appears  in  the  form  of  a  red  papule  with  indurated 
areola  in  five  or  six  days.  This  test  is  especially  valuable  in  tertiarj^ 
and  latent  syphilis,  conditions  in  which  the  Wassermann  reaction  is 
sometimes  negative.  It  is  valuable  also  in  differentiating  from  other 
conditions  which  might  give  a  positive  Wassermann,  for  this  test 
appears  to  be  a  specific  for  syphilis. 

Provocative  Wassermann. — This  test,  like  the  luetin  test,  is  valuable 
where  syphilis  is  suspected,  but  the  Wassermann  is  negative.  It  is 
dependent  upon  the  fact  that  a  negative  Wassermann  may  be  changed 
to  positive  after  an  injection  of  salvarsan.  This  change  may  occur 
quite  promptly  or  only  after  several  days.  Nichols  recommends 
making  the  Wassermann  twenty-four  and  forty-eight  hours  after  an 
injection  (0.4  gram  salvarsan  or  0.6  gram  neosalvarsan)  and  again 
after  seven  and  fourteen  days. 

Lange's  Colloidal  Gold  Test. — This  test  is  dependant  upon  a  color 
reaction  which  makes  it  very  delicate.  It  has  an  added  value  because 
a  single  test,  depending  upon  the  degree  of  discoloration,  tends  to 
show  whether  we  are  dealing  with  a  frank  luetic  or  a  metaluetic  pro- 
cess. For  this  reason  it  is  to  some  extent  replacing  the  Wassermann 
test.  For  a  description  of  the  technique  the  student  is  referred  to 
special  works.  A  number  of  other  tests  have  recently  appeared,  but 
those  mentioned  are  the  most  important. 

5.  The  Eye  Reflexes. — In  the  diagnosis  of  syphilis  of  the  nervous 
system  the  neurological  examination  of  the  eye  reflexes  is  of  para- 
mount value.  Here  irregularity  in  the  size  of  the  pupils,  irregularity 
in  the  pupillary  margins,  the  impairment  of  the  consensual  light 
reflex,  the  slowing  in  reaction  to  light,  fatigability  of  the  light 
reflex,  alterations  in  response  to  accommodation  efforts,  the  full 
development  of  the  Argyll-Robertson  syndrome  are  all  to  be  con- 
sidered. These,  one  or  all,  constitute  extremely  delicate  and  valuable 
criteria  for  the  clinical  appraisement  of  s\^philis  of  the  central  nervous 
system. 

A  fully  developed  Argyll-Robertson  syndrome — loss  of  direct 
pupillary  light  reflex,  with  free  and  ample  response  to  accommodation 
reflexes  in  one  or  both  eyes — represents  for  the  most  part  a  fairly  posi- 
tive criterion  of  syphilis  of  the  nervous  sj'stem. 

There  are  many  who  believe  that  this  syndrome  affords  positive 
proof  of  nervous  syphilis.  This  we  do  not  believe  to  be  true,  not  only 
upon  clinical,  but  also  upon  anatomical  grounds.  Clinicalh^  the 
Argyll-Robertson  syndrome  has  been  observed  following  direct 
injury  of  the  midbrain  structures  (pistol  shot — Guillain),  it  has  been 
observed  in  poisonings  other  than  those  of  syphilis,  alcohol  (in  Korsa- 
kow,  Wernicke's  polioencephalitis  superior).    It  may  result  from  press- 


DIAGNOSIS— PUPILLARY  SIGNS  521 

ures  (tumors  of  third  ventricle,  pineal),  from  poliomyelitis,  from 
trypanosomiasis,  and  from  other  rare  anomalous  disorders.  Anatomi- 
cally the  syndrome  represents  implication  of  certain  reflex  paths  in 
certain  peculiar  combinations,  and  such  implications  and  combina- 
tions are  purely  fortuitous  and  accidental,  i.  e.,  so  far  as  nosology  is 
concerned. 

As  a  matter  of  fact,  however,  these  combinations  rarely  take  place 
except  as  a  result  of  the  widespread  changes  induced  by  one  particular 
type  of  poisoning — the  syphilitic  virus — so  that  for  clinical  purposes 
the  presence  of  a  permanent,  bilateral,  Argyll-Robertson  syndrome  is 
nearly  enough  positive  for  syphilis  to  permit  one  to  assume  its  presence, 
and  to  therapeutically  guide  one's  self  accordingly  (Rose). 

Here  again,  however,  one  meets  with  the  pertinent  suggestion 
that  the  testing  for  the  Argyll-Robertson  syndrome  is  not  as  simple 
as  it  is  usually  supposed.  The  ordinary  devices  of  having  a  patient 
face  the  window,  and  then  cover  and  uncover  the  opened  eyes  with 
the  hand;  focusing  the  eye  upon  a  distant  object,  and  then  upon  the 
finger  in  close  proximity  to  the  nose;  these  tests  for  the  most  part 
are  entirely  too  crude  to  permit  one  to  judge  with  certainty  that  the 
Argyll-Robertson  syndrome  is  present.  Such  methods  may  suffice 
for  the  majority  of  instances,  but  in  no  field  of  neurology  is  it  more 
desirable  to  utilize  the  most  accurate  methods  than  in  dealing  with 
the  vexed  question  of  syphilis  of  the  nervous  system. 

Few  clinicians  can  follow  out  the  intricacies  of  Weiler's  complicated 
methods,  but  they  may  be  necessary  in  certain  doubtful  cases. 

Repeated  examinations,  under  carefully  regulated  supervision,  in 
the  daylight,  and  in  the  dark-room,  are  therefore  desirable  when  test- 
ing for  anomalies  in  the  pupillary  reflexes.  Careful  checking  of  the 
results  obtained  by  the  small  pocket  electric  lamps  is  imperative,  as 
occasionally  they  give  anomalous  results  and  may  lead  to  serious  errors 
(Oppenheim).  The  presence  of  a  fully  developed  Argyll-Robertson 
syndrome  may  be  said  to  be  a  highly  probable  positive  proof  of  syphilis 
of  the  central  nervous  system,  particularly  of  the  cerebral  and  mid- 
brain neurons.  Its  absence,  however,  by  no  means  negatives  syphilis 
of  the  nervous  system,  since  cerebral  gummas,  cerebral  syphilis, 
paresis,  tabes,  syphilitic  meningitis,  meningomyelitis,  cord  gummas, 
syphilitic  radiculitis,  syphilitic  neuritis,  all  may  be  present  without 
any  anomalies  in  the  pupillary  reflexes.  It  has  already  been  pointed 
out  that  the  laboratory  findings  of  paresis  and  of  tabes  have  antedated 
the  development  of  neurological  signs  by  at  least  a  couple  of  years, 
and,  moreover,  it  has  been  emphasized  that  pathological  alterations 
in  these  pupillary  reflexes  are  after  all  only  chance  happenings,  that 
certain  reflex  arcs  in  certain  combinations  are  caught  in  the  mesh  of 
the  infiltrative,  syphilitic  alterations.  The  chance  is  a  large  one,  it 
is  true,  but  still  it  is  purely  a  statistical  matter  of  what  has  happened. 
One  word  may  be  added:  in  many  cases  of  cerebrospinal  syphilis 
one  can  gauge  the  progressive  amelioration  of  the  patient's  condition 


522  SYPHILIS  OF  THE  NERVOUS  SYSTEM 

by  the  gradual  return  of  the  anomalous  pupillary  reflexes  to  a  more 
normal  condition.  Thus,  an  absolute  Argyll-Robertson  syndrome 
may  become  a  relative  one.  A  unilateral  Argyll-Robertson  may  be 
lost;  slowly  reacting  pupils  may  show  prompt  reactions,  irregularities 
in  size  may  disappear;  rapid  fatigability  may  let  up;  a  consensual 
light  loss,  often  the  first  anomaly  to  appear  in  cerebrospinal  syphilis, 
will  clear  up;  irregularities  in  the  pupillary  outlines  will  make  way  to 
regular  outlines,  etc. 

An  inability  to  modify  pathological  pupillary  reactions  by  ample 
syphilitic  therapy  argues  in  part  for  the  chronicity  of  the  process,  or 
the  inefiicacy  of  treatment.  This  is  not  an  absolute  rule,  however. 
It  may  be  possible  for  a  syphilitic  process  to  permanentl}^  destroy  por- 
tions of  the  pupillary  reflex  paths,  and  then  be  completely  and  perma- 
nently arrested.    The  pupillary  reflexes,  however,  remain  impaired. 

In  relation  to  this  question  of  the  pupillary  reflexes  and  anti- 
syphilitic  treatment  the  problem  arises.  What  is  the  probable  outcome 
of  a  syphilitic  process  which  comparatively  early  in  its  course  has 
destroyed  the  pupillary  reflex  paths  ?  Can  it  be  decided,  say  after  two, 
five,  or  ten  years,  during  which  time  there  has  existed  an  Argyll- 
Robertson  syndrome  and  little  else,  that  the  disease  has  been  com- 
pletely and  permanently  arrested?  Since  the  rapid  extension  of 
knowledge  concerning  the  cerebrospinal  fluid  this  question  can  be 
answered  better  at  the  present  time  than  ever  before. 

If  the  signs  of  a  meningitis — acute,  subacute,  or  chronic,  i.  e., 
increase  in  cellular  elements,  increase  in  globulin,  and  positive 
fluid  Wassermann,  remain  absent,  then  it  can  be  taken  as  highly 
probable  that  the  disease  process  has  been  arrested.  Judging  by 
clinical  methods  alone  a  long-standing  and  unchangeable  Argyll- 
Robertson  pupil  may  be  the  only  lesion  in  a  practically  cured  syphilis 
of  the  nervous  system.  It  is,  however,  to  be  realized  that  such  a 
pupillary  anomaly  may  exist  as  long  as  from  twelve  to  sixteen  years 
(alone)  and  then  the  patient  may  develop  a  paresis  or  tabes.  Of 
eleven  personal  observations,  now  existing  twenty  years,  only  two 
syphilitic  patients  with  long-standing  Argyll-Robertson  pupil  have 
not  developed  further  signs  of  brain  syphilis.  Certain  deep-seated, 
chronic,  syphilitic  arterial  processes,  which  may  lead  to  focalized 
lesions,  hemiplegia,  aphasia,  etc.,  may,  however,  go  on  for  some  time 
without  distinct  signs  of  meningeal  irritation  with  the  characteristic 
cellular  reactions. 

Clinical  Forms. — It  has  already  been  stated  that  the  clinical  forms 
of  syphilis  of  the  nervous  system  are  largely  abstractions.  The  patho- 
logical processes  are  predominantly  either  meningeal,  arterial,  infil- 
trative, i.  e.,  gummatous  in  character,  or  parenchymatous  alone  or  in 
combinations,  and  the  clinical  manifestations  are  extremely  variable, 
complex,  and  confusing,  depending  upon  the  interactions  of  the  patho- 
logical trends  and  the  variations  in  anatomical  paths  interfered  with. 

Fortunately  for  the  therapy,  the  clinical  type  is  of  secondary  con- 


SYPHILIS  OF  THE  CRANIAL  BONES  523 

sideration,  yet  there  are  certain  therapeutic  variables  that  render  it 
desirable  that  a  fidler  analysis  of  clinical  forms  should  be  made  than 
would  at  first  sight  seem  advantageous.  For  instance,  it  may  be 
recalled  that  certain  patients  with  meningeal  infiltrations  of  the  base, 
with  or  without  gummatous  nodules,  either  of  the  base  or  of  the 
convexity,  may  be  at  times  clinically  indistinguishable  from  a  paren- 
chymatous type.  A  nihilistic  therapeutic  attitude  relative  to  the 
latter  process  would  therefore  work  greatly  to  the  disadvantage  of  a 
patient  with  the  former.  A  like  situation,  though  perhaps  less  fre- 
quently, is  present  in  primary  syphilitic  endarteritis  of  the  vessels. 
That  type  of  syphilitic  endarteritis  of  the  small  vessels  of  the  cortex 
to  which  Alzheimer  has  called  particular  attention  is  another  case  in 
point. 

The  following  clinical  forms  are  to  be  distinguished: 

1.  Syphilis  of  cranial  bones. 

2.  Syphilis  of  the  basal  meninges. 

3.  Syphilis  of  the  convexity;  epilepsies. 

4.  Cerebral  syphilis — arterial  types. 

5.  Cerebral  syphilis — parenchymatous  types  (general  paresis,  tabo- 

paresis) . 

6.  Svphilitic  psychoses,  acute  and  subacute. 

7.  Tabes. 

8.  Syphilitic  spinal  meningitis;  meningomyelitis;  myelitis;    syphil- 

itic radiculoneuritis,  and  related  syphilitic  syndromes. 

9.  Hereditary  syphilis  of  nervous  system. 

For  a  fuller  consideration  of  the  protean  variations  the  monographs 
of  Riimpf,  Nonne,  Mott,  Plant,  Oppenheim,  Forster,  Schaffer,  and 
Fournier  should  be  consulted. 

1.  Syphilis  of  the  Cranial  Bones  Causing  Nervous  Symptoms. — 
These  were  recognized  as  early  as  the  end  of  the  sixteenth  century. 
Syphilis  of  the  cranial  bones  shows  itself  practically  in  the  form 
of  gummata.  Caries  of  the  cranial  bones  alone,  while  known,  rarely 
gives  rise  to  nervous  symptoms,  headache  excepted.  These  gummata 
may  be  circumscribed,  in  which  case,  if  large  enough,  they  give  rise 
to  the  symptoms  of  a  tumor  of  the  brain,  which  signs  are  largely  deter- 
mined by  the  precise  location  of  the  gummata.  These  circumscribed 
gummata  may  attain  enormous  proportions.  A  personal  observation 
(J.)  recalls  a  gumma  of  the  left  frontal  region  (the  size  of  a  tennis  ball) 
which  originated  in  the  bone  dura  and  protruded  into  the  right  frontal 
lobe.    Similar  gummata  are  not  infrequent. 

Cranial  bone  gummata — usually  involving  the  dura  as  well — are 
more  frequently  flattened  and  spreading.  Here  the  symptoms  of  brain 
tumor  are  usually  present.  Headache,  nausea,  vomiting,  sleepless- 
ness, are  among  the  general  symptoms,  while  localizing  signs  in  great 
variety,  depending  upon  the  situation  of  the  gummatous  masses 
are  present.  Epileptiform  convulsions,  lasting  for  years,  may  be 
the  sole  signs  of  such  gummatous  formations,  occupying  or  due  to 


524  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

pressure  upon  the  motor  areas.  Such  patients  are  often  mistakenly 
treated  as  epileptics,  and  the  monographs  of  Mott,  Oppenheim,  Riimpf, 
Nonne,  in  recent  years,  are  replete  with  autopsy  records  of  such  cases. 
Monoplegias  of  various  sorts  result  from  such,  as  also  aphasias,  word- 
blindness,  and  various  cranial  nerve  palsies. 

In  addition  various  forms  of  periostitis  and  of  osteitis  occur.  This 
latter  may  lead  to  exostoses  or  to  osteoporosis.  In  connection  with 
syphilis  of  the  vertebrae  it  should  be  remembered  that  the  processes 
are  more  often  involved  in  distinction  from  tuberculosis  that  more 
frequently  involves  the  body.  Syphilis,  too,  more  often  affects  the  cer- 
vical, while  tuberculosis  more  often  the  dorsal  vertebrae. 


Fig.  253. — Gumma  of  brain. 

Rarer  cases  of  cranial  bone  caries  of  the  base  (sphenoid)  compli- 
cated often  by  caries  of  the  upper  vertebrae,  are  also  known.  Petren 
has  studied  these  in  detail,  and  has  shown  the  value  of  the  a:-rays  in 
their  diagnosis. 

2.  Syphilitic  Meningitis  of  the  Base. — This  is  the  most  common 
form  of  cerebral  syphilis.  Its  most  frequent  site,  in  the  beginning,  is 
in  and  about  the  interpeduncular  space,  thus  almost  invariably 
involving  the  optic  chiasm.  From  here  it  tends  to  spread  in  all  direc- 
tions, pressing  into  the  sulci,  thickening  the  meninges,  by  infiltration, 
by  arterial  disease,  or  by  gummatous  growth.  Usually  all  types  of 
pathological  alteration  are  found.  The  gummatous  masses  not 
infrequently  invade  the  brain  structures  as  well,  grow  about  the  emerg- 
ing or  entering  cranial  nerves,  and  even  involve  the  bones  of  the  skull, 


SYPHILITIC  MENINGITIS  OF  THE  BASE  525 

and  the  upper  cervical  vertebrae.  Thus,  spinal  meningeal  infiltra- 
tions almost  invariably  accompany  this  basal  syphilitic  meningitis. 
Whereas,  the  diffuse,  conglomerate  types  are  more  frequent,  isolated 
vascular  disease,  circumscribed  gummata,  or  other  simpler  manifes- 
tations of  the  disease  may  occur,  in  which  latter  case  the  syndromes 
are  apt  to  be  simple. 

The  clinical  course  of  the  more  frequent  types  of  basal  syphilitic 
meningitis  often  resembles  general  paresis,  especially  in  the  beginning, 
but  the  gradual  extension  of  the  infiltrating  or  gummatous  develop- 
ments introduce  variants  which  often  permit  a  differential  diagnosis. 

Headache  is  a  frequent  and  early  sign,  often  preceding  other  symp- 
toms by  weeks,  months,  or  even  years.  It  has  the  frequent  nocturnal 
exacerbations  so  frequently  pictured  as  characteristic  of  syphilitic 
headache.  It  is  described  variously  as  boring,  stabbing,  and  percus- 
sion at  the  base,  may  show  tenderness,  though  less  frequently  than  in 
convexity  meningitis.  The  cervical  complications  spoken  of  often 
result  in  stiffness  of  the  neck. 

An  early  implication  of  the  optic  nerve  is  to  be  expected.  It  shows 
itself  (20  per  cent,  to  40  per  cent,  of  the  cases)  either  as  a  pressure 
neiu-itis  (neuritic  atrophy)  of  the  nerve  in  one  disk,  then  in  the  other, 
later,  if  headache  and  vomiting  or  other  signs  of  intracranial  pressure 
are  prominent,  choked  disk  in  both  eyes  is  apt  to  be  present.  Atrophic 
degeneration  and  optic  neuritis  are  less  often  found.  Marked  diminution 
in  visual  acuity  may  be  present  without  any  disk  evidences  of  disease. 

The  third  nerve  is  frequently  and  usually  irregularly  involved.  It 
is  characteristic  of  basal  syphilitic  meningitis  that  successive  branches 
are  implicated.  First  one  eye  may  show  a  ptosis,  then  perhaps  an 
internal  rectus  palsy,  then  the  other  eye  may  show  a  dilated  pupil, 
slow  in  its  reactions  to  light,  then  ptosis  develops  here.  Occasionally 
the  accommodation  reflex  is  lost.  A  series  of  cases  will  show  a  great 
variety  of  oculomotor  palsies.  A  true  Argyll-Robertson  syndrome  is 
not  infrequently  obtained. 

Other  cranial  nerves  are  often  included.  Variations  in  the  corneal 
reflex,  in  the  sensibility,  pain,  anesthesise  of  the  face,  point  to  a  tri- 
geminal complication.  A  peripheral  facial  palsy  may  be  present. 
In  some  individuals  the  deeper-lying  cranial  nerves  (IXth,  Xth,  Xlth, 
Xllth)  are  caught  in  the  syphilitic  extension,  with  their  characteristic 
symptoms.  The  eighth  nerve  is  probably  frequently  involved,  but 
often  too  slightly  to  elicit  complaint. 

The  mental  picture  is  usually  very  striking.  It  is  frequently  that  of 
a  slowly  developing  apathy,  or  heaviness  advancing  to  coma,  or  uncon- 
sciousness, with  periods  of  acute  confusion,  possibly  violent  delirium. 
There  is  a  marked  variability  in  these  patients  from  day  to  day  and 
also  considerable  differences  in  different  patients.  Some  patients 
develop  a  sort  of  drunken  delirium;  others  are  heavy  and  stupid  and 
apathetic;  others  are  furiously  violent. 

One  special  feature  is  frequently  met  with.    This  is  a  rapid  altera- 


526 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


tion  in  the  mental  picture,  when,  after  a  period  of  acute  confusion  or 
deep  coma,  the  patient  becomes  almost  practically  clear  within  a  few 
hours.  This  occurrence  may  even  follow  a  period  of  convulsive 
seizures.  Careful  tests  reveal  an  underlying  series  of  defects.it  is  true, 
but  from  a  lay  view-point  the  patient  may  appear  to  have  made  a 
complete  recovery  so  far  as  his  psychosis  is  concerned.  Without 
treatment,  however,  the  patient  again  develops  his  apathy,  confusion, 
delirium,  or  coma  and  not  infrequently  dies  in  this  state.  Sometimes 
death  results  by  suicide  as  the  patient  develops,  slowly  or  rapidly, 
a  distinct  depression  with  possibly  persecutory  ideas. 

Thus,  mentally,  the  patients  may  show  the  old-time  rubrics  of  acute 
confusion,  dementia,  mania,  melancholia,  paranoia,  etc.     This  alone 


Fig. 


254. — Cerebral  syphilis  ptosis. 
Third  nerve  palsy. 


Fig.  255. — Cerebral  syphilis  with  third 
and  fourth  nerve  palsies.     (Sharp.) 


indicates  the  futility  of  regarding  the  symptom  pictures  which  have 
gone  by  these  names  as  diseases,  some  for  hundreds  of  years.  Happily 
present-day  psychiatry,  largely  under  the  influence  of  Kraepelin's 
teaching,  recognizes  them  as  only  the  protean  and  kaleidoscopic  pic- 
ture-formation of  not  only  syphilis,  but  other  disease  processes  as  well. 

Biological  and  cytological  methods  have  permitted  this  definite 
change  in  attitude,  and  have  shown  the  essential  and  close  relation- 
ship of  many  diverse  neurological  and  psychotic  syndromes. 

3.  Syphilitic  Meningitis  of  the  Convexity. — This  differs  from  the 
former  only  in  the  trend  of  its  symptoms.  The  pathological  processes 
are  practically  identical.    Many  individuals  show  that  the  process  is 


SYPHILITIC  MENINGITIS  OF  THE  CONVEXITY  527 


['  Fig.  256. — Cerebral  syphilis  of  the  convexity.     Chronic  epileptiform  con^^ilsions. 


i.'i*^ 

*^;-.' 


:2k^ 


^    ii.. 


Fig.  257. — Syphilitic  meningitis  with  giant-cell  formation. 


528 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


general,  involving  both  the  base  and  the  convexity;  in  some  instances 
the  pathological  changes  being  more  marked  on  the  convexity  than  on 
the  base. 

Convexity  syphilis,  like  the  basal  variety,  may  be  a  fairl}^  localized 
affair,  or  it  may  be  diffuse.  It  may  be  limited  to  the  meninges,  or 
involve  the  bones,  or  the  brain,  or,  as  is  most  usual,  all  three. 

Here,  headache  is  a  prominent  sign.  It  is  paroxysmal,  and  often 
shows  a  nocturnal  increase  in  severity.  Percussion  affords  valuable 
evidence,  as  localized  tenderness  is  very  common. 

Here  the  general  syndrome  of  brain  pressure  is  usually  less  empha- 
sized. Nausea,  vomiting,  giddiness,  may  be  present,  but  are  usually 
late  in  development,  or  more  transitory.  Optic  nerve  changes  are 
less  frequent.    Isolated  symptoms  are  more  prominent.    Epileptiform 


Fig.  258. 


-Chronic  epileptiform  convulsions  due  to  syphilitic  leptomeningitis  and 
syphilitic  vascular  disease.      (Lafora.) 


convulsions  indicate  that  the  process  is  in  or  about  the  motor  areas. 
Not  infrequently  the  attacks  are  of  the  Jacksonian  type.  Involve- 
ment of  Broca's  convolutions  produces  temporary  or  more  enduring 
motor  aphasic  attacks.  Sometimes  these  aphasic  attacks  clear  up  in 
a  few  minutes,  an  hour  or  so,  or  a  few  days.  Minor  speech  difficulties 
may  only  indicate  the  possibility  that  an  aphasia  might  develop. 
Complete  motor  aphasia  develops  only,  as  a  rule,  with  hemiplegic  or 
monoplegic  accompaniments.  Pseudobulbar  palsy  attacks  indicate  a 
bilateral  involvement,  probably  both  cortical,  less  frequently  cortical 
on  one  side,  and  subcortical  on  the  opposite  side. 

]\Ionoplegias    of   varying   types    are    not   infrequent.      Bergmark^ 

1  Brain,  1911. 


CEREBRAL  SYPHILIS  529 

has  devoted  a  large  monograph  to  this  study.    Sensory  disturbances, 
hemianesthesise,  astereognosis,  haptic  hallucinations  are  met  with. 

With  diffuse  meningo-encephalitic  changes  the  picture  of  general 
paresis  is  assumed,  and  it  is  particularly  difficult  to  differentiate  this 
disorder.  The  clinical  pictures  may  be  as  various  as  those  of  paresis. 
Possibly  the  pnly  means  that  we  possess  to  distinguish  is  that  claimed 
by  Plant,  and  apparently  substantiated  by  Nonne  and  several  others, 
that  in  paresis  the  four  reactions  are  all  positive,  the  cerebrospinal 
fluid  showing  a  positive  Wassermann  with  0.05  c.c.  of  fluid.  With 
meningo-encephalitis  Hauptmann  and  Nonne  have  shown  that  the 
fluid  is  negative  when  small  quantities  are  used,  but  positive  when 
0.4  cm.  are  employed. 

This  generalization  seems  to  hold  true  not  only  for  convexity  menin- 
gitis, but  also  for  those  forms  in  which  the  base  is  more  especially 
involved,  although  as  will  be  later  pointed  out  in  the  discussion  of 
paresis,  variability  in  the  Wassermann  reaction  is  not  unusual. 

4.  Cerebral  Syphilis. — Vascular  Types. — In  considering  the  symp- 
tomatology of  patients  who  are  thought  to  have  cerebral  syphilis, 
attention  may  again  be  called  to  the  fact  that  the  dividing  lines 
between  cerebral  syphilis,  basal  meningeal  syphilis,  convexity  syphilis, 
etc.,  are  indefinite.  Rarely  does  one  find  a  pure  basal  or  convex 
meningitis  without  some  involvement  of  the  cerebral  substance  on  the 
one  hand,  while  conversely  it  is  as  rare  to  find  syphilitic  processes 
strictly  limited  to  the  cerebral  substance,  and  not  involving  the 
meninges.  One  can  postulate  pure  types  for  purposes  of  description, 
but  disease  is  rarely  a  respector  of  one  cerebral  tissue  more  than 
another. 

Symptoms. — It  is  for  this  reason  that  one  is  constantly  reminded 
of  the  multiplicity  of  symptoms  found  in  cerebral  syphilis.  In  this 
connection  it  would  not  be  without  profit  to  glance  for  a  moment  at 
the  diagnoses  of  certain  cases  reported  by  Nonne  in  his  monograph 
several  times  alluded  to.  The  patients  were  illustrations  of  basal, 
or  convexity,  or  encephalic  brain  syphilis,  usually  combined  forms. 
The  short  descriptive  diagnoses  run  as  follows:  Specific  headache  a 
year  after  infection,  with  secondaries  in  skin  and  mucous  membranes; 
headache  and  pupillary  anomalies;  headache  and  obstinate  vomiting, 
with  tertiary  testicle  signs;  progressive  simple  dementia  cured  by 
treatment;  progressive  dementia  with  defect;  combination  of  con- 
vexity meningitis  and  paresis;  gummatous  meningo-encephalitis  of 
convexity  with  general  symptoms,  choked  disk  and  antisyphilitic 
treatment  unavailing;  surgical  treatment  of  gummas  with  cure; 
Jacksonian  epilepsy;  cortical  epilepsy,  choked  disk,  paresis  of  left 
leg;  cortical  epilepsy,  optic  neuritis,  arterial  hemiplegia;  cortical 
hemiepilepsy  and  general  cortical  symptoms;  arterial  hemiapoplexy 
with  hemiepileptic  convulsions;  generalized  epileptic  seizures;  hemi- 
anesthetic  attacks  with  cortical  general  signs;  uremia,  etc.  Such 
illustrations  might  be  almost  indefinitely  continued.  They  are  not 
34 


530 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


the  exceptions,  they  are  the  rule.  One  is  tempted  to  indulge  in  the 
generalization  that  one  hundred  consecutive  patients  with  the  types 
of  cerebral  syphilis  under  consideration  would  show  one  hundred  dif- 
ferent clinical  syndromes.  Practically  all  of  those  just  enumerated 
belong  to  the  convexity  types  of  cerebral  syphilis.  Their  enumeration 
may  prove  of  service  in  localizing  the  process. 

A  similar  series  for  the  basal  types  may  be  equally  of  service: 
Gumma  of  right  frontal  lobe;  pressure  neuritis  of  optic  nerve;  choked 
disk  with  general  cerebral  symptoms;  bilateral  neuritic  optic  atrophy; 
recurrent  optic  atrophy;  hemianopsia;  bitemporal  hemianopsia; 
homonymous  left-sided  hemianopsia  with  right-sided  abducens  palsy; 
temporal    hemianopsia;  cranial    nerve    and    epilepsy;  hemianopsia; 


Fig.  259. — Syphilitic  meningitis.     Endarteritis  of  basilar  artery. 

hemianopic  pupillary  reaction,  oculomotor  palsy;  epilepsy,  oculomotor 
palsy;  ptosis;  internal  rectus  palsy;  optic  atrophy;  partial  oculo- 
motor palsy,  fifth,  seventh  palsy;  second,  third,  fourth,  fifth,  sixth 
nerve  involvement;  fifth,  sixth,  seventh,  eighth  nerve,  right  arm  and 
leg  palsy;  seventh,  eighth,  psychosis  (paranoia  acuta)  with  manic 
moods;  third,  fourth,  sixth,  tenth,  eleventh  nerve  palsies;  second, 
third,  fourth,  sixth,  seventh  with  epilepsy;  third,  fourth,  seventh, 
polyuria;  isolated  internal  ophthalmoplegia — to  mention  any  more 


CEREBRAL  SYPHILIS  531 

would  be  to  needlessly  extend  this  chapter.  The  lesson  such  findings 
indicate  is  obvious. 

But  eliminating,  as  far  as  possible,  the  varied  syndromes  of  cortical 
or  basal  syphilitic  meningitis,  and  Hmiting  the  discussion  of  the  present 
section  to  these  forms  of  cerebral  syphilis  due  more  particularly  to 
arterial  disease,  what  is  its  more  frequent  symptomatology? 

In  the  first  place  it  may  be  mentioned  that  arterial  types  of  brain 
syphilis  may  be  found  very  shortly  after  infection — as  short  a  time 
as  two  or  three  months.  Naunyn  in  a  thorough  study  found  that  48 
per  cent,  of  335  cases  reported  on  by  him  developed  signs  of  cerebral 
sj^philis  within  three  years.  On  the  other  hand,  forty  years  have  been 
known  to  elapse  between  infection  and  the  development  of  a  cerebral 
syphilis. 

Here  the  prodromal  symptoms  are  usually  headache,  dizziness, 
sleeplessness,  irritability,  inability  to  apply  one's  self  continuously 
to  one's  work,  lack  of  interest  in  work,  etc.;  in  general,  the  so-called 
neurasthenic  syndrome.    These  are  naturally  not  absolute. 

Headache. — The  headache  is  usually  very  disagreeable;  it  usually 
has  a  migratory  character — here,  there  and  elsewhere,  usually  dull, 
it  is  at  times  boring.  It  is  inconstant,  intermittent,  often,  not  by  any 
means  always,  worse  at  night  than  in  the  daytime.  It  may  disappear 
for  weeks  or  months,  and  then  suddenly  reappear.  It  may  also  be 
the  only  sign  of  cerebral  syphilis  for  months  or  even  years. 

Dizziness. — Dizziness,  in  shorter  or  longer  attacks,  is  very  insignifi- 
cant of  the  arterial  disease.  It  is  usually  associated  with  the  head- 
ache -but  may  appear  as  the  single  symptom  of  brain  syphilis.  Like 
the  headache  it  is  apt  to  be  increased  by  mental  or  physical  work. 

Insomnia. — Insomnia  is  frequent,  often  obstinate,  quite  variable 
and  not  infrequently  sleep  is  made  irregular  and  non-restful  by  the 
sense  of  heaviness  in  the  head  or  actual  headache. 

Psychical  Disturbances. — Psychical  disturbances  are  the  rule  in 
these  patients  with  arterial  disease  of  the  cerebrum.  They  become 
more  or  less  apathetic,  lose  interest  in  their  work;  are  unable  to  work 
because  of  forgetfulness  or  inefficiency.  With  this  there  is  increasing 
irritability,  an  inability  to  size  up  the  situation.  Such  severe  disturb- 
ance is  arrived  at  only  after  some  time  as  a  rule. 

Ahnormal  Sleepiness. — Abnormal  sleepiness,  coming  on  in  attacks, 
is  not  infrequent — such  periods  of  torpor  or  apathy  often  intermitting 
with  periods  of  anxiety  or  of  acute  restlessness  or  excitement.  Periods 
of  stupor  or  semidrunken  states  may  occur.  They  often  presage  more 
distinct  neurological  signs,  being  based  as  they  frequently  are,  upon 
sudden  extravasation,  infiltration  or  thrombotic  plugging  off  of  the 
blood  from  small  areas  of  brain  tissue. 

Many  patients  with  cerebral  syphilis  of  this  general  character 
remain  in  this  condition,  it  may  be  for  some  time;  the}^  show  a  picture 
precisely  similar  to  certain  patients  with  general  paresis.  It  is  in  this 
general  group  that  the  greatest  difficulties  in  diagnosis  occur.     As 


532 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


has  been  previously  stated,  a  positive  "four  reactions"  is  the  sole 
criterion  for  differentiating  the  two  in  the  present  state  of  our 
knowledge. 

Brain  tumor  is  also  to  be  thought  of  in  diagnosis.  Paresis,  brain 
tumor,  possibly  gumma,  cerebral  syphilis,  at  times  cannot  be  dis- 
tinguished one  from  another  clinically.  With  brain  tumor,  non- 
complicated by  syphilis,  the  absence  of  the  four  reactions  affords  a 
positive  criterion. 

Local  Symptoms.- — As  a  rule,  however,  the  greater  number  of  indi- 
viduals with  cerebral  syphilis  develop  local  symptoms,  and  neurologic 
rather  than  psychiatric  syndromes  come  into  relief,  or  the  latter  are 
intermingled  with  the  former.  Palsies  develop.  These  are  transi- 
tory, partial,  not  widespread,  or  may  be  severe,  complete,  and  per- 
manent, showing  various  hemiplegic 
syndromes,  according  to  the  anatom- 
ical site  of  major  disturbance — usually 
thrombotic — cortical,  capsular,  mid- 
brain, peduncles,  pons,  or  medulla — ■ 
the  symptomatology  of  the  different 
forms  of  which  are  dealt  with  else- 
where. 

Successive  attacks  of  mild  and 
transitory  palsies  are  very  signifi- 
cant of  cerebral  sj^philis.  Mono- 
plegias are  not  infrequent,  one  arm, 
one  leg,  one  side  of  the  face,  possi- 
bly the  cortical  speech  areas  with, 
in  case  of  double  lesion,  pseudo- 
bulbar palsy.  Minor  speech  dis- 
turbances are  extremely  frequent, 
tremors  of  the  facial  muscles  us- 
ually accompanying  the  stumbling, 
stuttering  or  drawling  speech. 

A  list  of  the  usual  clinical  diag- 
noses similar  to  those  already  outlined  for  basal  or  convexity  menin- 
gitis would  show  a  multiplicity  of  phenomena,  no  less  complex,  in  the 
neurological  field  and  certainly  infinitely  more  varied  in  the  mental 
symptom  pictures.  As  these  latter  will  be  discussed  more  in  detail 
in  the  section  on  the  psychoses  no  further  mention  will  be  made  of 
them  here. 

Parenchymatous  Form. — General  Paresis. — It  is  usual  to  separate 
paresis  as  well  as  tabes  from  other  syphilitic  disorders  of  the  nervous 
system,  under  the  general  caption  of  para-  or  metasyphilitic  disorders. 
Fournier  is  largely  responsible  for  this,  and  to  paresis  and  tabes  he  has 
added  a  number  of  other  disorders,  in  other  parts  of  the  body,  to  which 
he  applies  the  term  para-  or  metasyphilitic. 

Just  why  para-  or  metasyphilis  is  not   known,  especially  so  far 


Fig.    260. — Pseudobulbar  palsy  from 
syphilitic  disease.      (Tilney.) 


PARESIS 


533 


as  the  nervous  system  is  concerned.  Many  ingenious  hypotheses 
have  been  formulated  with  the  purpose  of  explaining  the  differences 
between  paresis  and  tabes,  on  the  one  hand,  and  other  forms  of  nervous 
syphilis,  secondary  or  tertiary,  on  the  other.  It  would  serve  little 
purpose  to  enumerate  them  in  detail,  since  none  has  as  yet  compelled 
conviction. 


Fig.  261. — Cerebral _  syphilis    (arterial    type)    with    softening.     Aphasia,    hemiplegia, 
advancing  dementia.      (Lafora.) 

To  return  to  the  syphilitic  etiology  of  paresis.  It  is  practically 
conceded  "no  syphilis,  no  paresis."  One  is  not  speaking  now  of  those 
few  individuals  who,  either  because  of  the  presence  of  brain  tumor, 
or  the  existence  of  arteriosclerosis,  or  of  other  cerebral  disorder,  show 
a  close  clinical  resemblance  to  paresis. 

Finally  the  findings  by  Moore  and  Noguchi  of  Treponema  paUidum 
in  twelve  of  seventy  paretic  brains  serve  to  render  more  certain  the 
relationship  of  the  organism  to  the  disease. 

But  syphilis  does  not  by  any  means  necessarily  lead  to  paresis,  for- 


534 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


tunately.  The  most  recent  studies  of  Mettler  show  that  about  2  per 
cent,  of  those  infected  by  syphiHs  develop  paresis.  A  considerably 
larger  percentage  develop  cerebrospinal  syphilis — how  large,  can  only 
be  surmised. 


Fig.  262. — Treponema  pallidum  in  the  brain  of  a  paretic.      (Moore.) 


The  question  then  arises,  How  is  it  that  in  certain  cases  a  disorder 
arises,  usually  more  than  five,  more  frequently  about  ten  years  after 
infection  which,  while  closely  resembling  many  forms  of  cerebral 
syphilis,  yet  differs  from  it  in  certain  very  noteworthy  particulars, 
and  what  underlies  these  differences?  In  other  words,  Why  para- 
or  metasyphilis  ? 


PARESIS 


535 


Naturally  there  are  those  who  say  there  is  no  difference,  either 
anatomically,  biologically,  or  therapeutically.  They  are  in  the  mi- 
nority with  certain  well-developed  arguments,  some  of  which  are  as 
yet  unanswerable.  The  present-day  attitude  is  to  maintain  a  dis- 
tinction between  the  strictly  cerebral  syphilitic  disorders  and  general 
paresis,  chiefly  because  the  histological  pathology  is  unique,  the 
biological  tests  are  dift'erent,  and  the  results  of  therapy  diverse. 


Fig.  263. — Ammon's    horn   in    paresis.     Pyramidal-cell   layer   has  disappeared,   vessels 

infiltrated. 


Alzheimer  and  Nissl  have  laid  down  fundamental  distinctions  in 
the  pathological  picture.  The  chief  points  are  quantitative,  and  to 
a  less  extent  qualitative.  In  paresis  the  parenchymatous  changes 
are  predominant;  in  syphilis  the  vascular.  Their  researches  have 
remained  uncontro verted,  although  modified. 

Symptoms. — The  syndrome  is  characterized  by  a  bewildering  mul- 
tiplicity  of  forms,   which,   shifting   in  the  individual  patient   from 


536  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

month  to  month,  at  tunes  even  from  day  to  day,  prevent  any  clean- 
cut  description  that  will  embrace  its  many  vagaries.  Attempts  have 
been  made  to  create  empirical  types.  Thus,  Binswanger,  in  a  notable 
study,  created  three  types  based  on  certain  anatomical  peculiarities — 
meningitic-hydrocephalic,  hemorrhagic,  and  taboparetic  forms.  Such 
an  anatomical  subdivision  has  no  reliable  clinical  counterparts. 

Attempts  at  the  erection  of  clinical  types  are  perhaps  slightly  more 
encouraging,  for  there  are  certain  patients  who  will  run  a  course  true 
to  type. 

The  earlier  monographs  of  Simon  and  Kraftt-Ebing,  and  the  later 
ones  of  Joffroy,  Obersteiner,  Klippel,  and  Kraepelin^  suggest  the 
following  groups: 

1.  Simple  dementing  types. 

2.  Simple  depressed  types. 

3.  The  expansive  or  so-called  classical  type. 

4.  The  agitated  types. 

5.  The  irregular  types  with  localized  symptoms,  Lissauer,  tabo- 
paretic form. 

6.  Juvenile  paresis. 

Before  even  attempting  a  description  of  these  purely  artificial 
creations,  pictures  which  are  constantly  shifting  and  showing  com- 
binations of  details,  a  brief  glance  at  the  chief  symptom  components 
is  advisable. 

These  have  frequently  been  divided  into  the  mental  and  physical, 
but  as  this  is  a  purely  arbitrary  distinction  it  will  not  be  emphasized 
here.  As  has  been  noted,  a  diagnosis  of  an  impending  paresis  may  be 
made,  at  times  some  years  before  its  onset,  by  the  findings  in  the 
cerebrospinal  fluid,  but  attention  is  here  first  focussed  upon  the 
mental  picture.  A  peculiar  psychical  weakness  is  one  of  the  early 
phenomena.  A  difficulty  in  perceiving  external  impressions  shows 
this  intellectual  loss.  In  the  early  stages  it  may  require  special  study 
of  reaction  times,  which  are  usually  lengthened,  but  soon  absent- 
mindedness,  inattention,  loss  of  details,  forgetfulness  of  important 
facts,  become  apparent.  There  is  a  gradually  developing  loss  of 
ability  for  prolonged  mental  effort;  in  conversation  finer  shades  of 
meaning  are  lost,  the  patient  is  no  longer  alert  and  keen,  as  perhaps 
has  been  his  normal  habit.  The  mental  deterioration  going  on 
leads  to  many  changes  in  his  usual  conduct,  until  the  patient  may 
be  no  longer  quite  sure  of  himself  in  his  customary  surroundings. 

Certain  patients  develop  a  state  of  dreamy  consciousness,  as  though 
in  a  mildly  intoxicated  state. 

Increased  fatigability  is  another  early  symptom.  Much  has  been 
written  of  the  preneurasthenic  stages  of  paresis.  This  excessive 
fatigue  may  prevent  him  from  starting  anything  new — sometimes 
he  even  falls  asleep  while  at  work  or  in  conversation. 

1  General  Paresis,  Nervous  and  Mental  Disease  Monograph  Series,  No.  15. 


PARESIS 


537 


Defects  of  Retention  and  Memory. — Retention  and  memor}^  soon 
commence  to  show  defects.  Careful  studies  in  the  early  stages  have 
shown  difficulties  in  association,  lessened  capacity  for  learning,  dis- 


FiG.  264. — Paresis.     Cortical  changes.     Epileptiform  convulsions.     (Lafora.) 

turbance  in  attention,  often  with  good  retention.     The  patients  for- 
get recent  happenings  more  readily,  not  knowing  what  has  transpired 


Fig.  265. — Paresis  with  Korsakow  syndrome;  Wernicke's  polioencephalitis  superior. 


a  week  ago,  yesterday,  sometimes  a  half-hour  ago.  These  grosser 
defects  belong  to  the  later  stages  as  a  rule.  The  memory  of  time  rela- 
tions gradually  slips  away,  the  patient  being  unable  to  arrange  sue- 


538  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

cessive  phases  in  an  orderly  series.  Thus,  many  of  these  patients 
show  the  greatest  defects  in  their  appreciation  of  time  differences, 
when  married,  age  of  oldest  child,  and  related  striking  facts  of  life. 
In  later  stages  all  sense  of  time  may  become  effaced. 

Impoverishment  of  Ideas. — A  gradual  impoverishment  of  ideas  takes 
place,  varying  in  degree  from  month  to  month  and  showing  also  great 
variability  with  different  patients.  Those  associations  most  in  use 
usually  persist  the  longest,  and  all  tests  should  take  into  consideration 
special  aptitudes.  In  the  later  stages  the  patients  have  lost  prac- 
tically all  of  their  mental  possessions — the  gap  at  times  being  filled 
in  by  retrospective,  confabulatory  reminiscences.  Certain  of  the 
patients  resemble  patients  with  a  Korsakow  syndrome. 

Loss  of  Judgment. — Loss  of  judgment  naturally  goes  on  pari  passu 
with  the  general  psychical  disintegration.  In  the  early  stages  even 
refined  methods  of  testing,  such  as  those  of  Gregor,  show  that  uncer- 
tainties, contradictions,  logical  lapses  are  not  infrequent.  The  patients 
are  easily  distracted  by  sound  associations,  9x9  =  99,  etc.  As  this 
loss  of  judgment  goes  on  the  patients  may  make  the  most  absurd 
plans.  They  do  the  most  unusual  things,  often  involving  their  entire 
fortunes  and  playing  havoc  with  all  of  their  carefully  raised  social 
fabric.  Dream  world  and  real  world  become  hopelessly  confused  in 
this  fundamental  psychic  crumbling. 

Hallucinations,  illusions,  and  changes  in  simple  sensory  perception 
are  found,  but  they  are  not,  as  a  rule,  prominent  features  in  paresis. 
Delusion  formation  is  naturally  present  in  many  instances,  although 
certain  patients  may  go  through  the  disorder  with  but  few  delusional 
developments. 

Delusions. — The  delusional  ideas  vary  immensely — they  are  usually 
senseless  and  fantastic  and  when  combined  with  active  creative  phan- 
tasies, as  they  frequently  are,  especially  in  agitated  or  excited  periods, 
pass  all  bounds.  These  patients  think  in  millions,  billions,  quadrillions, 
etc.  They  are  princes,  kings,  emperors,  potentates,  priests,  Christ, 
god,  supergods.  They  have  rubies,  pearls,  diamonds,  emeralds;  two 
wives,  a  dozen,  a  harem,  thousands  of  beautiful  women,  etc. 

These  delusional  ideas,  simple  or  phantastic,  are  also  liable  to  great 
lability.  They  are  always  changing;  contradictory  as  well  as  uncon- 
scious. New  ones  come,  old  ones  go,  revivals  take  place.  Progres- 
sions may  go  backward :  Now  they  have  millions,  next  moment  have 
thousands;  now  a  king,  in  ten  minutes  a  fine  soldier.  One  can  at 
times,  by  talking  with  these  patients,  expand  or  contradict  their 
delusional  exuberance  almost  at  will. 

The  same  characteristics  may  be  noted  in  regard  to  delusions  of  a 
depressive  or  hypochondriacal  nature.  Nihilistic  delusions  such  as 
believing  they  have  no  stomach,  no  heart,  or  are  dead  are  not  infre- 
quent in  these  types. 

AUeraiion  of  Emotional  Activity  .—The  disposition  or  emotional 
reactivity  is  involved,  as  is  the  intelligence.     As  a  rule  the  patients 


,    PARESIS  539 

in  the  early  stages  are  hyperexcitable — others,  however,  are  markedly 
depressed.  They  are  apt  to  be  touchy,  surly,  cross,  even  having  violent 
outbursts  for  the  most  trivial  events.  There  is  often  a  distinct  damper 
in  their  higher  ethical  feeling,  so  that  the  stimulus  of  conversation, 
the  joy  of  music  or  art,  of  various  social  relations  gives  way  to  a 
careless  indifference,  often  at  great  variance  with  the  psychomotor 
activity  of  the  patient. 

As  the  disorder  progresses  the  mood  is  apt  to  be  colored  by  the 
delusional  interpretations.  Anger  and  laughter  may  follow  one  another 
in  quick  succession,  and  a  great  variety  of  fleeting,  changeable,  often 
contradictory,  emotional  states  are  passed  through. 

Character  Alterations. — The  character  alterations  are  predominant. 
Decision  is  progressively  lost;  instability  and  foolhardiness  alter- 
nating with  obstinacy  and  perverseness.  Initiative  is  reduced,  and 
the  patient  may  become  as  clay  in  the  potter's  hand;  such  periods 
often  alternate  irregularly  with  impulsive  heedlessness.  Kraepelin 
relates  the  case  of  a  patient  who  stepped  out  of  a  second-story  window 
to  pick  up  a  cigar  that  he  happened  to  notice  on  the  walk  beneath  him. 
Criminal  actions  may  be  committed  in  just  the  same  manner  as  the 
case  of  the  paretic  who  shot  at  Mayor  Gaynor,  of  New  York.  Suicide 
may  occasionally  take  place  in  the  same  manner.  Stealing  is  by  no 
means  infrequent,  and  sexual  misdemeanors  and  crimes  are  extremely 
prevalent.  This  blunting  of  the  repressions  inculcated  by  the  force 
of  civilization  is  particularly  noticeable,  and  predominantly  in  the 
sexual  sphere.  Hence  results  the  frequent  telling  of  lewd  stories, 
consorting  with  people  of  quite  inferior  social  status,  exhibitionism, 
shameful  and  open  masturbation,  and  even  sexual  assaults. 

Neurological  Signs. — Here  one  finds  not  infrequently  in  the  begin- 
ning phases  a  dull,  heavy  headache.  Hyperacusis  often  precedes  the 
blunting  of  the  special  sense,  and  various  localized  disturbances,  such 
as  word  blindness,  word  deafness,  auditory  hallucinations,  apraxia, 
asymbolia,  astereognosis,  indicate  a  special  localization  for  the  time 
being  in  more  or  less  definite  cortical  areas.  Optic  nerve  atrophy 
occurs,  at  times  early,  in  from  5  to  10  per  cent,  of  the  cases.  Special 
changes  in  the  optic  disk  are  recognizable  in  from  12  to  50  per  cent, 
of  the  cases. 

Changes  in  Cutafieous  Sensibility. — Very  frequently  cutaneous  sen- 
sibility is  modified — sharp  pains,  numbness,  itching,  etc.,  occur,  and 
in  those  forms  recognized  as  taboparetic  these  often  show  the  special 
localizations  of  the  tabetic.  Out  of  these  changed  sensations  delu- 
sional interpretations  frequently  arise.  A  general  insensibility  to 
peripheral  stimuli  develops  later,  and  the  patient  may  then  pay  little 
attention  to  any  kind  of  irritant,  heat  or  cold,  full  bladder,  distended 
rectum,  etc.  Occasionally  such  patients  mutilate  themselves,  cutting 
off  a  finger,  or  the  tongue,  or  the  testes  in  order  to  get  rid  of  what  seems 
to  them  a  foreign  body. 


540 


SYPHILIS  OF  THE  NERVOUS  SYSTEM 


Motor  Incoordinations. — Motor  incoordinations,  from  initial  trem- 
bling to  more  high-grade  ataxias,  apraxias,  adiadochokinesias,  Rom- 
berg, asynergias,  are  common.  Intention  tremor  is  not  infrequent, 
and  perseveration  is  almost  never  missed  in  the  later  stages. 

Disorders  of  Speech.— In  speech  these  inotor  difficulties  have  been 
specially  studied  since  Esquirol  first  laid  stress  upon  such  changes  in 
mental  cases.  Frequently  beginning  with  slight  stumbling,  a  slurring 
over  certain  letters  or  syllables,  r,  /,  etc.,  the  paretic  develops  very 
characteristic  speech  anomalies.  These  come  out  with  marked  promi- 
nence in  the  use  of  test  phrases — electricity,  Methodist  Episcopal, 
organization,  truly  rural,  third  cavalry  brigade,  etc. — when  certain 
letters  are  repeated,  stumbled  over,  or  elided.  Paraphasia,  aphasia, 
ataxia  of  speech,  perseveration  are  among  the  frequent  later  develop- 
ments until  only  a  mumbling  may  be  possible  in  some  of  the  last 
stages.    Similar  changes  take  place  in  writing. 


Fig.  266. — Set  of  pictures  of  paretic  convulsion.     (Kraepelin.) 

Eye  Symptoms. — In  the  ocular  movements,  analogous  difficulties 
are  observed  and  in  the  pupils  one  observes  a  variety  of  changes. 
Statistical  studies  show  these  pupillary  anomalies  to  be  extremely 
frequent.  Differences  in  size  from  50  to  80  per  cent.  (Rache);  dis- 
tortion of  the  pupillary  outlines,  74  per  cent.  (Joffroy) ;  Argyll-Robert- 
son pupil,  50  to  70  per  cent.  (Westphal,  Junius,  Arndt,  etc.).  Many 
of  these  pupillary  anomalies  undergo  considerable  variation,  changing 
from  time  to  time  even  without  treatment.  Loss  of  consensual  light 
reflex,  as  already  noted,  is  often  one  of  the  earliest,  and  at  the  same 
time  one  of  the  most  persistent  of  the  pupillary  anomalies. 

Convulsive  Phenomena. — Convulsive  phenomena,  epileptiform  or 
apoplectiform  in  character,  are  rarely  missed  in  paresis.  They  are 
usually  of  the  cortical  epileptic  type.  They  often  occur  early  in  the 
disease  or  may  punctuate  any  period  in  its  development.  At  times 
limited,  they  more  often  are  generalized,  and  frequently  have  pro- 
dromata,  such  as  dreamy  states,  motor  incoordination,  thickness  of 
speech,  twitchings,  etc.,  as  a  rule  occurring  early.  As  isolated  phe- 
nomena, typical  status  attacks  may  be  observed,  with  as  many  as  100 
or  more  epileptiform  crises  in  twenty-four  hours.    An  attack  in  the 


PARESIS  541 

very  early  stages  may  last  only  a  few  seconds;  the  patient  suddenly 
sinks  back  on  his  chair,  and  is  all  right  in  a  few  moments — while,  on 
the  other  hand,  status  attacks  may  persist  a  week  or  even  more. 
Unconsciousness  is  usual,  though  it  may  be  very  slight  or  fleeting. 
A  vast  variety  of  focal  residuals  have  been  described. 

Similar  changes  may  be  observed  on  the  sensory  side  of  the  nervous 
system,  and  so-called  psychic  equivalents,  as  in  the  more  classical 
epilepsies,  are  frequent. 

Statistical  studies  show  the  very  great  frequency  of  these  attacks, 
Obersteiner  recording  them  as  often  as  in  90  per  cent,  of  his  patients; 
while  Junius  and  Arndt  in  their  recent  extensive  study  give  them  as 
occurring  in  53  per  cent.  A  personal  study  (J.)  of  two  hundred  cases 
showed  them  in  78  per  cent,  of  the  patients.  Kraepelin  believes 
that  treatment  in  bed  limits  the  number  and  frequency  of  the  attacks; 
his  Munich  statistics  show  an  incidence  of  about  65  per  cent. 

Alterations  in  Reflexes.- — The  tendon  reflexes — triceps,  radius^ 
knee-jerks  and  Achilles — are  usually  positively  involved,  either 
excessive,  in  the  greater  number  of  cases,  or  diminished,  especially  in 
those  patients  with  posterior  cord  involvement,  which  is  frequent. 
When  the  deep  reflexes  are  found  to  be  increased  other  sjinptoms  of 
involvement  of  the  pyramidal  tracts  are  not  infrequent.  Babinski 
reflex,  very  frequently  Chaddock's  external  malleolar  sign,  at  times 
the  paradoxical  reflex  of  Gordon  occlu's.  Possibly  there  is  an  ankle 
clonus,  and  spasticity  in  gait  is  present.  If,  on  the  other  hand,  the 
deep  reflexes  are  diminished,  other  signs  of  involvement  of  the  posi- 
tion sense  and  deep  sensibility  fibers,  travelling  the  posterior  column 
pathways,  are  usually  found.  Ataxia,  Romberg,  girdle  sensations, 
anesthesise,  etc.,  pains  of  the  radicular  type,  are  also  often  encountered 
in  these  taboparetics. 

In  most  of  the  patients  there  is  great  variability  in  the  two  sides. 
Occasionally  one  finds  spasticity  of  one  and  hypotonia  and  ataxia  of 
the  other,  and  combined  symptoms  are  to  be  expected  in  the  later 
stages,  especially  in  those  patients  with  prominent  cord  localizations. 

In  the  final  stages  contractures  occur  in  the  bed-ridden  patient. 
They  are  unable  to  do  anything  and  muscular  twitches,  spasms, 
localized  atrophies,  and  a  veritable  museum  of  anomalies  is  to  be 
looked  for. 

Findings  in  Cerehrospijial  Fluid. — The  findings  in  the  cerebrospinal 
fluid  have  already  been  discussed.  Suffice  it  to  say  here  that  they  are 
of  paramount  importance  and  a  diagnosis  of  paresis  without  the  signs 
obtainable  in  the  cerebrospinal  fluid  must  always  be  regarded  as 
lacking  in  a  most  important  element. 

Enough  has  been  said  to  show  that  the  clinical  picture  of  paresis 
may  be  closely  counterfeited  by  a  number  of  other  pathological  states 
- — notably  brain  tumor,  cerebrospinal  syphilis,  arteriosclerosis,  chronic 
alcoholism,  sleeping  sickness,  etc. 

The  findings  in  the  fluid  are  very  definite.    A  positive  fom-  reactions. 


542  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

the  fluid  used  in  small  quantities — 0.05  to  0.2  c.c. — is  almost  certainly 
diagnostic  of  paresis,  yet  at  times  it  would  appear  that  positive  four 
reactions  are  found  in  other  syphilitic  processes  which  do  not  behave 
like  paresis.  The  earlier  didactic  attitude  of  Plant  seems  to  be  in 
need  of  some  revision,  but  at  the  present  time  there  is  not  sufficient 
autopsy-controlled  material  to  permit  absolute  dicta. 

Vasomotor  and  Troyhic  Disturbances. — Vasomotor  and  trophic  dis- 
turbances may  appear  early  and  come  and  go;  among  them  skin  erup- 
tions, such  as  herpes,  and  pemphigus  are  the  commoner  types  met 
with.  The  ready  appearance  of  bed-sores  and  abscesses  indicate  the 
lowered  resistance  of  the  skin  and  subcutaneous  structures. 

The  bodily  temperature  may  show  considerable  variation,  even  on 
opposite  sides  of  the  body;  it  is  usually  subnormal  in  the  later  stages, 
save  following  convulsive  seizure.  Sleep  is  irregular,  especially  in 
the  excited  stages,  when  the  paretic  may  not  sleep  for  days — whereas, 
in  torpid  stages  or  in  those  quiet,  dementing  forms  the  patient  sleeps 
or  is  in  a  doze  much  of  the  time. 

The  appetite  is  capricious,  and  the  bodily  weight  is  apt  to  fall  off 
in  the  early  stages  and  during  excitement,  to  become  much  increased 
in  the  torpid,  quiet  states. 

Disorders  of  the  bladder,  and  incontinence  of  urine  and  feces,  all 
sooner  or  later  come  within  the  outlines  of  the  picture. 

Remissions. — One  clinical  feature  which  is  very  striking  is  the 
tendency  of  this  disorder  to  show  marked  remissions.  In  certain 
respects  this  is  a  general  law  in  disease  processes,  but  in  paresis  it 
appears  most  striking  because  of  the  almost  miraculous  change  that 
takes  place  in  the  patient.  Such  patients  one  would  say  were  about 
to  die;  they  become  absolutely  helpless,  convulsion  follows  convul- 
sion; in  the  interim  they  know  nothing,  are  bed-ridden,  have  to  be 
fed,  soil  themselves,  and  are  reduced  simply  to  breathing,  heart-beat- 
ing automata.  They  may  remain  in  this  condition  for  weeks  and 
months,  and  then  pick  up  a  little,  and  then  more  and  more,  and  within 
a  space  of  six  weeks  to  three  months  many  such  patients  appear  to 
be  almost  well  and  like  themselves.  They  have  risen  from  the  dead, 
and  strange  to  say,  although  the  relatives,  friends,  and  business  asso- 
ciates have  been  told  over  and  over  again  perhaps,  for  they  should 
be,  that  this  is  not  a  cure,  that  it  is  only  a  remission  of  symptoms,  the 
patient  is  frequently  restored  to  all  his  civil  rights  and  given  full  con- 
trol of  his  affairs.  In  the  majority  of  cases  this  is  disastrous;  he  may 
launch  out  into  new  lines,  involve  his  fortune,  marry  unwisely,  and 
then  after  a  few  months,  perhaps  a  year — the  longer  remissions  on 
record  have  been  five  or  six  years — the  average  in  about  six  months — 
the  symptoms  return,  often  in  rapid  progression,  and  usually  lead  to 
death  after  variable  intervals  of  from  six  months  to  a  few  years. 

Forms. — To  return  now  to  the  subject  of  the  forms — those  more 
or  less  artificial  groups  which  for  the  purposes  of  description  psychia- 
trists areagreed  upon. 


PARESIS— FORMS 


543 


The  symptomatology  of  paresis  varies  within  such  wide  hmits 
because  of  the  extent  and  distribution  of  the  pathological  changes; 
because  of  the  individual  make-up  of  the  patients^  and  because  the 
disease  process  not  only  affects  the  highest  psychological  levels  but 
strikes  deep  into  the  physico-chemical  and  sympathetic  foundations 
upon  which  these  higher  levels  are  built.  The  disease  presents, 
therefore,  a  combination  of  psychological  symptoms  more  or  less 
explainable  at  that  level  coupled  with  disintegrations  of  a  much  more 
material  character  and  stable  organization. 

1.  Dementing  Form. — That  which  characterizes  this  general  group 
is  the  progressive  mental  deterioration  with  motor  paresis.     Excite- 


FiG.  267. 


-Simple  dementing  form  of 
paresis. 


Fig.   268. — Paresis.     Demented  type 
of  facies. 


ments,  convulsions,  extravagant  delusion  formations  are  not  prominent 
and  when  present  are  transitory.  In  these  individuals  there  is  the 
early  period  of  nervous  irritable  weakness,  with  loss  of  mental  alert- 
ness, moodiness,  inability  to  work,  forgetfulness,  and  steadily  increas- 
ing poverty  of  thought.  Naturally,  the  picture  beginning  in  this  way 
may  suddenly  change.  This  is  sufficient  to  throw  the  patient  into 
another  group — but  if  the  development  is  of  the  slow,  progressive 
nature,  gradually  advancing  mental  weakness,  fleeting  delusional 
ideas,  often  with  childish,  weak-minded  features,  these  are  the  general 
symptoms  of  the  dementing  type  of  paresis. 

2.    Depressed  Forms. — Here  anxious  depression  is  in  the  foreground 
of  the  mental  picture.    Hypochondriacal,  delusional  states  are  promi- 


544  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

nent.  The  patients  continually  complain  about  bodily  discomfort; 
have  lost  their  intestines,  or  have  destroyed  their  manhood  by  mas- 
turbation or  sexual  excesses.  These  delusional  ideas  become  more 
and  more  nonsensical.  In  many  instances  the  hypochondriacal  ideas 
are  dependent  upon  fancied  sinful  actions  or  wrong-doing.  They  are 
great  sinners,  they  must  be  protected  from  the  police,  they  fear  they 
will  be  sent  away.  Such  patients  often  have  persecutory  ideas,  and 
when  such  are  prominent  early  in  the  disease,  before  there  is  marked 
deterioration,  they  are  frequently  regarded  as  "paranoiacs"  especially 
by  those  schools  which  regard  names  as  disease  entities  and  seek  for 
diagnoses  from  a  "pathognomonic"  symptom.  These  paretics  with 
persecutory  ideas  often  have  pronounced  hallucinations  of  hearing. 

Notwithstanding  these  hypochondriacal  or  persecutory,  delusional 
interpretations,  these  patients  are  markedly  indifferent;  they  are 
apathetic,  talk  and  move  in  a  monotonous,  dull  manner,  and  take 
little  interest  in  their  surroundings. 

Thus  the  loss  of  energy,  the  libido  in  Jung's  sense,  not  being  able  to 
go  forward  in  the  affairs  of  life,  a  marked  pathological  introversion 
takes  place,  and  the  regression  takes  hold  of  all  sorts  of  nonsensical, 
childish,  infantile,  and  archaic  phantasies.  Pathological  projection 
also  is  common,  and  one  has  a  regular  chaos  of  pathological  mental 
mechanisms.  The  sense  of  reality  is  so  markedly  impaired,  and  the 
affective  relativity  so  cut  off,  that  the  nonsensical  beliefs  have  no 
corresponding  or  adequate  emotional  relationships.  The  personality 
is  fragmenting  and  disintegrating. 

Childish  regressions  of  hero  formation  appear.  The  patient  is  a 
god,  a  king,  an  emperor;  like  Jack  and  the  beanstalk,  he  is  miles 
high;  as  in  the  Lilliputian,  he  is  a  great  giant;  as  in  Midas'  touch,  or 
Aladdin's  lamp,  he  breaks  the  bank  at  Monte  Carlo,  or  is  the  owner 
of  immense  gold  mines,  fabulously  valuable  jewels,  etc. 

Contrasting  states  of  great  inferiority,  weakness,  poverty,  cause  them 
to  be  very  fearful,  easily  confused,  easily  lost;  they  beg  for  protection, 
hide  from  anger,  or  ask  piteously  for  food,  preserve  scraps,  etc. 

They  become  unmanageable  in  bed,  and  finally  in  many  the  agita- 
tion and  fear  develop  great  resistance  and  violence.  Self-destruction 
may  be  attempted,  mutilation  occasionally  occurs.  Most  of  these 
attempts,  however,  are  fragmentary,  non-sustained  and  bungling. 

Stuporous  states  show  a  contrast  to  this  marked  violence.  They 
may  persist  for  weeks,  months,  or  even  years.  The  patients  lie  stupidly, 
"depressed,"  or  anxious  in  bed,  unclean  and  unmanageable.  Special 
rigidities,  catatonic-like  in  their  nature,  may  develop. 

The  special  statistics  show  that  from  15  per  cent,  to  20  per  cent, 
of  the  material  in  some  of  the  larger  European  hospitals  and  clinics 
may  be  in  general  thrown  into  this  depressed  category. 

3.  Expansive  Types. — This  general  type  has  been  for  years  con- 
sidered "classical,"  yet  they  are  not  as  frequent  as  the  demented  types. 
Thus,  Kraepelin  gives  30  per  cent,  in  his  Heidelberg  series,  Junius 


PARESIS— FORMS 


545 


and  Arndt  27  per  cent,  in  their  Berlin  material.  It  is  to  be  regretted 
that  the  specialist  has  failed  to  emphasize  this  feature,  which  is  of  so 
much  value  to  the  general  practitioner,  and  has  confused  the  issues  by 
speaking  of  a  "change  in  type."  It  is  of  more  value  to  insist  upon  the 
comparative  rarity  of  the  megalomanic  features  of  paresis,  since,  as  the 
average  medical  man  has  been  taught  to  recognize  paresis  by  this  sign, 
it  is  not  to  be  wondered  at  that  so  much  delay  has  occurred  before 
the  recognition  of  paresis.  The  emphasis  should  not  be  laid  upon 
the  comparatively  rare  expansive  cases. 

In  this  megalomanic  type  the  boastful  ego  rises  to  superior  heights. 
Everything  is  seen  from  the  stand-point  of  a  feeling  of  abundant 
energy.  At  first  the  ideas  are  those  of  great  exaltation,  within  the 
bounds  of  normal  human  experience,  but  soon  the  patient  loses  his 
earthly  bonds  and  soars  to  superhuman 
unrealities.  His  strength  is  appalling; 
his  education  superior  to  any  others 
in  the  world;  he  speaks  ten,  nay,  all 
languages;  has  all  wealth;  all  power; 
figures  mount  from  thousands  to  mil- 
lions, to  pages  of  ciphers.  And  in  kalei- 
doscopic changes,  and  great  individual 
variation  one  learns  of  many  marvels 
of  superior  excellence  only  dreamed  of 
in  childish  phantasy,  or  seen  in  the 
boasts  of  inferior  peoples. 

One  feature  of  this  frightful  megalo- 
mania, which  has  its  very  great  ups 
and  downs,  should  never  be  over- 
looked; namely;  the  tendency  for  such 
patients  to  commit  sexual  indiscre- 
tions, even  atrocities;  or  to  engage 
in  the  most  foolhardy  enterprises, 
thus  jeopardizing  life  and  property. 

This  feature  in  paresis  is  of  so  much 
importance  that   special   attention   should  be  devoted   to  the  legal 
measures  which  should  be  invoked  to  prevent  the  worst  consequences 
of  this  mental  weakness. 

That  megalomania  has  a  distinct  deterioration  background  is  seen 
in  the  frequent  combination  of  a  poor  clerk,  in  a  state  institution, 
who  speaks  of  the  million-dollar  novel  he  is  writing.  It  consists  of  a 
few  miserable  scrawls  on  toilet  paper,  or  on  the  edges  of  a  daily  news- 
paper. This  is  only  a  type.  Such  inconsistences  may  be  read  of  in 
the  classics  of  psychiatry,  from  the  work  of  Arnold,  in  1700,  to  the 
present  time. 

These  phantastic,  exalted,  euphoric  states  very  frequently  elaborate 
on  sexual  themes.     Thus  the  patients  have  hundreds,  millions  of 
wives  or  concubines — "Solomon  was  a  piker  in  this  matter,"  boasted 
35 


Fig.   269. 


Paresis,   showing  gran- 
diose tj'pe. 


546 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


a   Bellevue  patient.     The   children   are  more   numerous   and  more 
beautiful  than  any  promised  to  the  ancient  Hebrew  heroes. 

One  patient,  mentioned  by  Kraepelin,  could  lift  ten  elephants,  was 
two  hundred  years  old,  9  feet  tall,  was  a  beautiful  Adonis,  weighed 
four  hundred  pounds,  had  an  iron  chest,  an  arm  of  silver,  a  head  of 
gold,  100  wives,  1000  million  boys  and  girls,  his  urine  was  Rhine  wine, 
and  his  feces  were  gold. 


Fig.  270. — Excited  paretic.      (Kraepelin.) 

The  illustrations  might  be  repeated  ad  infinitum.  They  are  to  be 
found  in  richer  or  poorer  elaboration,  in  shorter  or  longer  intervals  of 
excitement,  in  this  exalted  euphoric  type,  but  one  may  see  a  hundred 
paretics,  as  a  general  practitioner  may  see  them,  in  the  early  stages, 
and  never  get  a  ghost  of  an  idea  of  such  experiences.  Of  this  100, 
sometime,  sooner  or  later,  25  to  30  of  them  will  be  liable  to  exhibit 
the  exalted,  euphoric,  megalomanic  picture  here  indicated  rather 
than  described. 


PARESIS— FORMS  547 

Consciousness  is  usually  much  clouded  in  this  type,  especially  while 
the  delusional  projections  are  in  their  full  growth.  Time,  place,  the 
great  world,  is  a  dreamy,  far-off  world  of  little  moment  to  the  mind 
engaged  in  its  ambitious  program.  Continuity  of  thought  is  practi- 
cally impossible,  and  chaos  and  anarchy  exist.  In  such  minds  hallu- 
cinations are  frequent. 

The  mood  is  happy,  overflowing  with  schemes  for  good  deeds  and 
generosity,  and  all-embracing  in  its  brotherly  love.  But  coherence 
is  not  to  be  expected.  Hypochondriacal  ideas,  such  as  delusions  that 
there  are  worms  in  the  head  may  rest  in  bizarre  connection  with  the 
delusion  of  being  a  great  philosopher,  a  Shakespeare,  etc.,  and  changes 
in  mood  are  of  frequent  occurrence.  Weeping  follows  ecstasy,  and  is 
replaced  by  beatific,  sublime  happiness.  Sudden,  passionate  excite- 
ment leaps  up  under  restraint,  to  subside,  or  to  be  diverted  by  such 
a  trifle  as  a  falling  leaf,  or  a  ring  at  the  door  bell. 

The  great  psychomotor  excitement  is  a  striking  feature,  and  one 
difficult  to  manage.  These  patients  walk  miles,  are  on  the  go,  meeting 
people,  busily  engaged  in  everybody's  business,  making  plans  for  self 
and  others,  and,  when  confined,  the  limits  of  a  paretic's  violence 
knows  no  bounds.    He  is  transformed  into  a  raving  animal. 

Throughout  all  of  the  excitement,  divertibility,  constant  changing 
of  plans,  mixture  of  silly  pleasure  and  superficial  sadness,  there  is  the 
note  of  great  deterioration  of  critique  and  emotional  degradation  and 
deterioration  which  shows  particularly  in  the  conventions  relative  to 
one's  person.  Carelessness  in  dress,  uncleanliness,  grossness  in  eating, 
loss  of  finer  susceptibilities,  coarse  expressions,  frank  immoralities^ — ■ 
these  are  but  a  few  of  the  possibilities  in  such  lax  conduct. 

In  watching  such  patients  from  day  to  day,  one  is  struck  by  the 
immense  variability  in  the  picture.  The  ideas  of  grandeur  may  all 
vanish,  the  patient  denies  he  ever  said  any  such  thing,  he  may  get 
angry  in  a  dispute  over  the  matter,  and  then  launch  into  a  magnificent 
grandiloquent  invective  of  colossal  outlines. 

As  the  dementia  increases,  these  large  ideas  may  entirely  disappear, 
or  be  preserved,  and  appear  on  the  surface  only  as  a  few  words,  or 
murmurs,  "good  to  eat,"  "fine  women,"  "millions,"  etc. 

Finally  in  the  later  stages,  the  patients  all  sink  to  a  more  or  less 
common  level — "sans  everything." 

Among  the  expansive  forms  may  be  found  the  quick,  galloping  cases 
who  die  within  a  short  time.  Increasing  experience  seems  to  show, 
however,  that  these  excited  types  indicate  a  very  severe  reactive  pro- 
cess, and  hence,  if  they  do  not  die  in  the  height  of  the  reaction  (gallop- 
ing cases),  they  provide  the  greater  number  of  the  more  stationary 
and  protracted  forms — ^those  who  make  a  partial  recovery  with  defect, 
and  who  later  disintegrate.  Remissions  seem  to  be  common  in  this 
type  as  well. 

4.  Agitated  Foriiu. — Those  patients  who  show  a  predominant  motor 
activity  in  the  beginning  may  be  said  to  be  grouped  here.     Great 


548 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


restlessness  runs  through  the  entire  picture.  The  mental  content  is 
very  variable — euphoric,  depressed,  hypochondriacal,  mood  colora- 
tions flit  in  and  out.  Galloping  cases  are  usually  grouped  here,  in 
which  an  extremely  rapid  and  fatal  course  is  present. 

This  is  really  only  a  subgroup  of  the  preceding  type,  only  artificially 
separated  off  by  reason  of  the  more  consistently  persistent  psychomotor 
restlessness.  Remissions  are  frequent,  as  are  also  the  apoplectiform 
and  epileptiform  attacks.  The  pathological  process  simply  has  a  wider 
extension  in  the  motor  areas. 

The  acute  delirious  cases,  somewhat  resembling  delirium  tremens 
of  alcoholism,  and  independent  of  it,  are  arranged  by  Kraepelin  in  the 
agitated  group. 

5.  Irregular  Types. — Lissauer,  etc.  These  patients,  showing  irregu- 
lar forms  of  development;   neurosyniptomatic  groupings,  hemiplegias, 


Fig.  271. — Paresis  with  tabetic  changes  in  spinal  cord. 

etc.,  are  here  brought  together.  The  hemiplegic  and  taboparetic 
groups  are  the  more  frequent. 

Taboparesis.- — ^Taboparesis  is  the  more  striking  of  these  irregular 
forms  and  deserves  a  further  outlining. 

It  has  been  assumed  by  many,  especially  by  neurologists  (Schaffer, 
for  summary,  1912)  that  tabes  may  be  regarded  as  a  spinal  paresis,  and 
paresis  a  cerebral  tabes;  that  is,  the  disease  varies  only  by  reason  of 
the  greater  severity  of  the  process  in  the  one  or  the  other  localization. 

Kraepelin,  on  the  other  hand,  accentuates  the  opposing  psychiatric 
view,  that  whereas,  the  two  disorders  are  undoubtedly  fundamentally 
syphilitic,  yet  they  are  two  different  kinds  of  processes,  and  that  when 
the  symptoms  of  tabes  are  added  to  paresis  the  changes  in  the  cord  are 
not  exactly  similar  to  those  found  in  tabes  limited  to  the  cord.  The 
different  findings  in  the  cerebrospinal  fluid  in  the  two  disorders  would 
point  to  some  sort  of  a  difference  as  well. 


JUVENILE  PARESIS  549 

The  matter  still  rests  in  the  lap  of  the  gods. 

Clinically,  taboparesis  shows  in  a  combination  of  the  symptoms 
observed  in  the  two.  forms.  Raymond  and  Nageotte  would  have  it 
that  every  paretic  would  show  tabetic  signs  if  he  lived  long  enough. 
In  those  patients  with  pronounced  tabetic  onset  one  finds  the  frequent 
pupillary  anomalies,  the  diminution  or  loss  of  the  patellar  reflexes, 
Romberg  sign,  ataxia  of  lower  or  upper  extremities,  or  both,  hypo- 
tonia, lancinating  pains,  crises,  and  arthropathies.  These  are  found 
in  the  more  definite  tabopareses  closely  associated  with  the  psychical 


Fig.  272. — Juvenile  paresis  in  the  emaciated  stage. 

disturbances  already  outlined.  In  the  more  classical  tabetic  patients 
the  mental  disturbances,  to  which  Cassirer  and  0.  Meyer  have  devoted 
their  attention,  are  very  distinct  from  those  of  paresis. 

6.  Juvenile  Paresis. — This  form  is  quite  distinct.  It  was  apparently 
first  recognized  as  late  as  1877  by  Clouston.  It  appears  at  the  present 
time  not  infrequently,  since  the  Wassermann-Plaut  findings  offer  such 
certain  criteria  for  its  determination.  Such  tests  seem  necessary, 
since  the  clinical  picture  may  be  so  extremely  variable — hence  it  was 
overlooked — many  patients  dying  diagnosed  as  "imbeciles." 


550 


SYPHILIS  OF  THE  NERVOUS  SYSTEM 


Here  the  patient  may  make  a  comparatively  normal  development 
to  five  or  ten  years  of  age — certain  non-developmental  forms  probably 
belong  here,  but  are  now  disregarded.  Then  the  child's  mentality 
seems  to  drop.  In  older  children,  ten  to  sixteen,  this  drop  is  more 
apparent.  Poor  memory,  bad  motor  adaptation  and  gradual  demen- 
tia appear.  Childishness,  fabrication,  excitements  and  depressions, 
fears,  and  anxieties,  are  frequent.  Epileptiform  convulsions  appear 
— many  juvenile  paretics  are  gathered  into  the  almshouses  and  epi- 
leptic colonies  as  "epileptics  with  feeble-mindedness" — and  after  a 
course  of  three  or  four  years,  with  gradually  deepening  mental  disin- 


FiG.  273. — Brain  of  a  patient  with  a  syphilitic  psychosis  of  acute  maniacal  type. 
Enlarged  and  engorged  vessels.     Syphilitic  meningitis. 

tegration,  the  patient  dies.  The  histopathological  changes  are  identical 
with  those  of  the  adult  form. 

It  is  noteworthy  that  the  age  of  onset,  from  seven  to  twelve  years, 
is  the  same  length  of  time  that  in  the  adult  form  elapses  between 
infection  and  the  outcrop  of  the  metasyphilitic  disease. 


SYPHIUTIC  PSYCHOSES. 

In  this  section  are  included  the  psychoses  which  are  associated 
with  cerebral  syphilis  and  with  tabes.  In  the  present  state  of  our 
knowledge  we  are  not  prepared,  as  already  stated,  to  make  a  clear 
distinction  either  on  pathological,  clinical,  or  psychological  grounds 
between  the  so-called  metasyphilitic  and  the  more  clearly  syphilitic 


SYPHILITIC  PSYCHOSES 


551 


m£ 


:i^- 


Fig.  274. — Brain  of  a  patient  with  a  chronic  syphiUtic  psychosis  of  maniacal  type. 
Syphilitic  leptomeningitis,  pachymeningitis. 


'%  t^ '- 


Fig.  275. — Brain  of  juvenile  paretic  showing  marked  atrophy. 


552  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

conditions,  and  there  are  undoubtedly  all  sorts  of  gradations  between 
these  two  practical  divisions. 

Forms. —  Neurasthenia. — Kraepelin  speaks  of  a  syphilitic  neuras- 
thenia— a  form  of  mental  disturbance  much  written  upon  by  earlier 
authors.  The  preneurasthenic  phase  of  a  cerebral  syphilis  or  of  a 
paresis  is  not  now  under  review.  It  is  apt  to  appear  shortly  after 
infection  and  manifest  itself  in  a  nervous  discomfort,  difBculty  in 
thinking,  irritability,  disturbance  of  sleep,  pressure  in  the  head, 
variable  and  changeable  discomfort  and  pain.  To  these  may  be 
added  slight  depression,  dizziness,  confusion,  anxiety,  slight  difficulty 
in  finding  words,  temperature  variations,  paresthesise,  and  nausea. 

Many  students  prefer  to  interpret  these  symptoms  as  a  direct  result 
of  the  infection,  and  not  as  a  circumscribed  syndrome;  but  slight 
vascular  changes,  minute  pupillary  alterations,  and  particularly  evi- 
dence of  meningeal  irritation  as  shown  by  the  spinal-fluid  lympho- 
cytosis, point  in  the  direction  of  its  being  something  more  than  a 
simple,  infectious  reaction. 

Plant  has  described  the  psychoses  which  are  associated  with  cerebral 
syphilis  and  with  tabes  in  ten  groups.    The  groups  are  as  follows : 

1.  Simple  Luetic  Weakness  of  Mind. — ^This  is  the  weakness  of  mind 
which  usually  goes  with  gross  lesion  of  the  brain,  marked  by  hemiplegia 
or  monop^legia.  It  is  generally  the  result  of  the  blocking  of  a  consider- 
able vessel  by  a  thrombotic  process  and  usually  occurs  in  relatively 
young  people.  There  is  no  well-defined  type  of  mental  defect  resulting, 
as  this  is  dependent,  of  course,  upon  the  location  of  the  lesion  and  upon 
the  make-up  of  the  individual.  There  may  be  depression  or  euphoria 
or  a  simple  forgetfulness  and  indifference.  Occasional  cases  of  arterio- 
sclerosis occurring  early  in  life  simulate  this  condition  very  closely. 

2.  Syphilitic  Pseudoparesis. — Here  we  have  a  group  of  cases  which 
seem  to  occupy  all  portions  of  the  territory  between  the  true  syphilitic 
psychoses  and  paresis.  On  the  mental  side  the  distinction  between 
pseudoparesis  and  paresis  is  practically  impossible  to  make.  Persistent 
auditory  hallucinations,  however,  seem  in  experience  to  have  pointed 
quite  strongly  to  pseudoparesis.  The  most  reliable  diagnostic  criterion 
is  the  behavior  of  the  cerebrospinal  fluid  toward  the  Wassermann 
reaction,  it  being  often  negative  in  vascular  syphilis  and  positive  in 
paresis.  While  this  is  not  an  absolute  differentiation  and  larger  doses 
of  the  serum  may  produce  the  positive  reaction,  it  is  stiU,  however, 
one  of  the  most  important  differentials.  It  has  to  be  remembered,  too, 
that  some  cases  of  paresis  are  found  with  negatively  reacting  fluid, 
and  rarely  cases  of  lues  with  positively  reacting  fluid. 

3.  Paranoid  Forms  Combined  with  Tabes. — In  this  group  are  found 
patients  who  do  not  show  any  considerable  deterioration,  but  present 
ideas  of  persecution  with  numerous  auditory  hallucinations  over  a 
considerable  period  of  time.  There  is  no  self-reproach,  they  remain 
lively  and  affable,  and  what  seems  to  be  peculiar,  present  marked 
hallucinations  of  common  sensibility  accompanied  by  phantastic  ideas. 


SYPHILITIC  PSYCHOSm 


553 


4.  Paranoid  Forms  Without  Tabetic  Symptoms. — In  this  group  are 
found  paranoid  ideas  combined  with  auditory  halhicinations,  rather 


Fig.  276. — Devastation  of  cortex  in  paresis.     (Kraepelin.) 

resembUng  the  alcohoHc  halhicinoses.    A  pronounced  delusion  of  jeal- 
ousy was  present  in  one  case  described  by  Plaut.    They  have  to  be 


554  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

separated  from  manic-depressive  psychosis  and  particularly  from  de- 
mentia precox.  The  separation  from  the  latter  is  made  from  the  absence 
of  catatonic  signs  and  failure  to  develop  marked  evidences  of  defect. 

5.  Certain  Epileptic  Forms. — These  appear  to  be  due  to  the  endar- 
teritic  changes  in  the  small  cortical  vessels  and  may  be  combined  with 
paralytic  phenomena  which  develop  as  a  result  of  the  participation  of 
the  larger  vessels  in  the  disease  process.  The  cases  resemble  very 
closely  genuine  epilepsy  and  must  be  differentiated  by  the  neurological 
and  serological  findings.  A  case  described  by  Plant  showed  transitory 
dream  states. 

6.  Short  Hallucinatory  Confused  States. — ^These  resemble  the  con- 
fusions associated  with  the  epileptic  forms,  and  the  French  have 
considered  them  in  the  same  class  with  the  crises. 

7.  Psychotic  Disturbances  Associated  with  Syphilitic  Cardiac  Disease. 
— ^This  condition  probably  develops  most  frequently  in  connection 
with  syphilitic  aortitis. 

8.  Psychoses  Resembling  Manic-depressive  Pyschosis. — ^Here  con- 
ditions are  grouped  which  superficially  very  closely  resemble  the  manic- 
depressive  psychosis.  As  a  rule,  however,  there  is  something  to  attract 
attention  as  indicating  at  least  an  aberrant  form.  On  the  mental  side 
the  delusions  are  more  grotesque,  more  out  of  harmony  with  the 
personality  of  the  patient,  or  show  an  unreasonableness  which  is  not 
commensurate  with  the  degree  of  excitement.  On  the  physical  side, 
of  course,  inactive  pupils  should  lead  to  a  serological  examination. 
Occasionally  such  episodes  occur  a  long  time  before  the  outcrop  of 
frank  symptoms  of  metasyphilitic  disease. 

9.  Mental  Disorder  Due  to  Syphilis  as  a  Psychic  Trauma. — Here 
a  psychogenic  psychosis  which  is  more  apt  to  take  a  depressive  form 
is  included. 

10.  Hereditary  Luetic  Mental  Disturbances. — ^This  envisages  psycho- 
pathically  defective  subjects  and  weak-minded  children  with  luetic 
etiology.  The  exact  relation  between  lues  and  various  forms  of  weak- 
mindness  is  not  accurately  known,  but  it  is  known  that  a  large  number 
of  the  feeble-minded  group  are  luetic.  Syphilitic  brain  disease  may 
occur  in  early  infancy  and  proceed  for  some  time,  producing  only 
transient  symptoms,  perhaps  an  occasional  convulsion,  and  ulti- 
mately lead  to  serious  defect. 

TABES. 

History. — In  any  historical  presentation  a  sharp  distinction  must  be 
made  between  the  name  tabes  dorsalis,  and  the  disease  as  now  under- 
stood. So  far  as  is  known,  the  former  had  its  origin  with  Hippocrates, 
the  latter,  if  one  accepts  the  Post-Columbian  origin  of  syphilis, 
could  have  come  into  existence  among  Europeans  and  their  descendants 
after  the  Sixteenth  century  only. 

The  various  interpretations  given  to  the  Hippocratic  term  through- 


TABES 


555 


out  the  ages  is  a  chapter  of  surprises.  Spermatorrhea,  gonorrhea, 
leucorrhea,  gleet  were  its  initial  meanings,  with  or  without  signs  of 
organic  disease  of  the  cord;  when  combined  with  cord  signs — mye~ 


Fig.  277. — Ganglion-cell  degeneration 
and  rod  cells  in  paresis.     (Lafora.) 


^  •*«*,.. 


iif. 


'     1^ 


J  t  - 


\^ 


Fig.  278. — Axis-cylinder  degeneration  in 
paresis.     (Kraepelin.) 


litic  processes,  usually  tuberculosis— Pott's,  etc.— it  was  called  tabes 
nervosa  or  myelophthisis.  Out  of  this  mass  the  disease  of  the  present 
day  was  separated.    It  was  natural  that  excessive  venery  should  have 


556 


SYPHILIS  OF  THE  NERVOUS  SYSTEM 


been  attributed  as  the  cause.    As  late  as  the  early  part  of  the  last 
century  (1804)  the  term  persisted  in  the  sense  of  a  "nervous  weak- 


FiG.  279. — Brain  of   a  paretic  showing  marked   atrophy.     Flaking  of  the  cortex  due 

to  adhesions  of  the  pia. 


Fig.  280. — Organization  of  Virchow-Robin  lymph  spaces  in   general   paresis.     Tannin 

silver.     (Achucarro.) 


TABES 


557 


ness,"  i.  e.,  what  today  one  would  designate  a  "sexual  neurasthenia," 
or  a  "sexual  phobia."  So  far  as  can  be  made  out  from  Litt're's  Hippo- 
crates this  is  exactly  what  Hippocrates  wrote  about  under  the  term 
tabes  dorsalis.  Even  in  the  theses  of  Harles  and  Brera,  in  the  early  part 
of  the  nineteenth  century,  the  relation 
between  the  cord,  the  vessels  of  the 
lower  abdomen,  and  secretions  from  the 
genito-urinary  organs  was  held  to  be  a 
close  one. 

As  to  the  earliest  observations  of 
modern  tabes,  the  traces  are  very  in- 
distinct. To  the  clinicians  of  the  six- 
teenth and  seventeenth  centuries,  the 
confused  mass  of  paraplegias  was  prac- 
tically insoluble,  and  it  would  appear 
that  it  was  only  in  the  beginning  of  the 
nineteenth  century  that  the  process  of 
differentiation  took  place.  E.  Horn 
(1816)  called  attention  to  a  number  of 
the  important  features  including  blind- 
ness; Weidenbach  (1817)  attempted  to 
make  a  separate  disease  of  it — he  con- 
tested the  inflammatory  origin — said 
it  had  nothing  to  do  with  consumption, 
although  still  unable  to  break  away 
from  the  belief  in  the  excessive  venery 
etiology  then  rampant.  Schesmer  (1819) 
described  the  peculiar  gait  in  an  unmis- 
takable manner,  while  W.  Horn  (1827) 
emphasized  the  real  absence  of  a  true 
paralysis,  and  spoke  of  an  ataxia  where- 
by this  affection  was  different  from  other 
forms  of  myelitis.  Decker  (1838)  called 
attention  to  the  swaying  and  unsteadi- 
ness with  closed  eyes,  which  was  taken 
up  by  Romberg  three  years  later  and 
rechristened  Romberg's  sign. 

Pathologically  the  characteristic  cord 
signs  were  not  unobserved.  Hutin 
(1828)  describes  them,  Ollivier  of 
Anglers  (1837)  gave  the  picture,  re- 
produced here  in  part,  while  Cruveilhier  (1832-1845),  in  his  Atlas, 
gives  masterly  clinical  and  pathological  descriptions. 

Romberg  in  the  first  edition  of  his  Lehrbuch  gave  greater  precision 
to  the  description,  and  Steinthal  (1847)  threw  together  the  incom- 
plete paralysis  (or  ataxia),  the  Romberg  sign,  and  the  characteristic 
gait,  but  without  any  real  grasp  of  the  situation  clinically  or  patho- 


FiG.  281. — Illustration  of  the  cord 
of  a  tabetic  given  by  Ollivier  of 
Angiers  in  1837. 


558  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

logically.  Later  Romberg,  in  the  second  edition  of  his  text-book 
(1851)  gave  greater  precision  to  the  concept,  and  gave  a  classical 
description.  Finally,  Duchenne,  in  the  years  1852  to  1858,  elaborated 
the  general  idea,  and  gave  the  first  complete  and  adequate  description 
of  the  disorder.  It  may  be  said  that  Horn  and  Romberg  had  practi- 
cally made  out  of  the  general  tabes  dorsalis  collection  a  special  tabes 
dorsalis  collection  in  the  sense  of  our  present-day  conceptions  in  the 
physiological  and  pathological  fashioning  of  which  Tod  (1847),  who 
seemed  to  grasp  the  fact  that  incoordination  and  posterior  columns 
were  related,  Rokitansky  (1854),  Virchow  (1855),  Turck  (1856), 
Landry  (1858),  and  Gull  (1858)  made  lasting  contributions.  The 
recent  history  gives  us  the  names  of  Charcot  (1868),  Vulpian,  and 
Topinard,  and  in  1863  three  monographs  appear  by  Eisenmann,  Ley- 
den,  and  Friedreich,  while  the  later  contributions  of  Westphal  (1875), 
Argyll-Robertson  (1869),  Marie,  Redlich  and  Obersteiner,  Nageotte, 
Oppenheim,  Dejerine,  Goldscheider,  Erb,  Nissl,  Schaffer,  and  Alzheimer 
record  the  detailed  studies  in  etiology,  symptomatology,  pathogenesis 
and  spinal  cord  conditions  of  the  last  two  decades.^  Finally  the 
even  more  recent  work  of  the  serologists,  particularly  Wassermann, 
Citron  and  Plaut,^  and  Noguchi  have  said  the  final  word  regarding 
the  etiological  factor,  syphilis. 

Etiology  and  Occurrence. — Syphilis  is  the  only  cause  of  tabes.  The 
statistical  method  had  almost  proved  the  syphilitic  etiology  of  tabes, 
but  with  the  advent  of  the  objective  methods  of  Wassermann  and  his 
students,  all  doubts  have  vanished.  In  the  more  recent  work,  of  the 
most  competent  serologists,  syphilitic  antibodies  are  found  in  the 
blood  serum  of  practically  every  case  (Plant,  loc.  cit.).  Furthermore, 
the  cytological  examination  of  the  cerebrospinal  fluid  shows  the 
presence  of  cellular  exudates  characteristic  chiefly  of  the  syphilitic 
processes,  and  as  will  be  seen  in  the  consideration  of  the  pathological 
features;  the  syphilitic  nature  of  many  of  the  findings  is  beyond 
controversy.  Finally  Treponema  pallidum  has  been  found  in  the 
spinal  cord  areas  in  tabetics. 

Pseudotabetic  syndromes  are  known  to  occur  in  multiple  sclerosis,  in 
tumor,  in  caries,  in  poisoning  by  alcohol,  pellagra,  diabetes,  ergot,  etc. 

What  secondary  factors  are  necessary  to  determine  why  this  or 
that  patient  infected  with  syphilis  should  develop  tabes  cannot  yet  be 
answered.  Less  than  one-half  of  1  per  cent,  of  the  infected  develop 
the  disease,  and  other  factors  are  demanded  on  a  priori  grounds  to 
explain  why  one  syphilitic  individual  develops  it  and  99  syphilitics 
do  not;  and  this  is  true  only  for  certain  races.  The  many  cases  of 
congenital  tabes,  following  a  syphilis  from  the  same  source,  suggest 

1  Jelliffe,  On  Some  of  the  More  Recent  Literature  of  Tabes  Dorsalis,  Pathology  and 
Etiology,  International  Clinics,  1907,  ii,  257.  Later  literature,  see  Schaffer,  Lewandow- 
sky  Handbuch  and  work  by  Massary,  1909. 

2  The  Serodiagnosis  of  Syphilis  in  Psychiatry  (Nervous  and  Mental  Disease  Mono- 
graph Series,  1911). 


TABES  559 

variations  in  the  virus.  Similarly  the  large  number  of  tabetics  who 
are  known  to  have  followed  the  wake  of  certain  syphilitic  prostitutes 
(Morell,  Lavalee,  Erb,  Brosius — glass-blower  cases).  The  other  factor 
must  be  due  to  variations  in  resistance. 

Both  of  these  factors  are  impossible  to  measure,  but  it  is  highly 
doubtful  if  any  of  the  many  causes  usually  mentioned,  such  as  ex- 
posure to  cold  and  wet,  trauma,  excessive  venery,  etc.,  have  anything 
to  do  with  the  after-development  of  a  tabes.  The  multiplicity  of 
assigned  causes  makes  it  more  than  probable  that  none  are  concerned. 
Ford  Robertson's  specific  bacillus  is  certainly  not  a  proven  secondary 
factor.  Trauma  is  highly  doubtful.  It  may  cause  a  pseudotabes, 
or  may  hasten  the  fuller  development  of  the  symptoms.  Of  heredity 
little  is  known.  Charcot,  Borgherini,  Erb,  and  Gowers  have  laid  much 
stress  upon  it. 

Occurrence. — The  majority  of  cases  occur  in  the  fourth  decade,  but 
this  is  largely  due  to  the  fact  that  the  disorder  comes  on  ten  to  twenty 
years  after  infection,  and  syphilitic  infection  usually  occurs  between 
the  twentieth  and  thirtieth  years.  The  disease  may  appear  at  almost 
any  period  after  infection  (fifteen  to  seventy  years  of  age).  The 
average  runs  from  thirty-five  to  forty,  the  average  interval  after 
infection  about  fifteen  years,  with  extremes  at  four  to  thirty-five. 
Dejerine  and  Raymond  report  cases  forty-five  and  fifty  years  after 
infection.  In  certain  races  with  a  high  syphilis  percentage  (Algiers) 
tabes  is  practically  unknown,  and  there  is  every  reason  to  believe  that 
the  syphilis  has  been  there  present  since  the  sixteenth  century.  In 
other  races,  from  three  to  five  of  every  one  hundred  syphilitics  develop 
the  disease.  In  certain  countries  the  ratio  of  men  to  women  is  4  to  1, 
in  others  10  to  1. 

Occupation  seems  to  play  only  that  role  that  speaks  for  increased 
opportunities  for  syphilitic  infection. 

Symptoms. — The  symptomatology  of  tabes  is  markedly  diverse, 
and  whereas  it  seemed  at  one  time  that  it  presented  a  more  regular 
picture  than  other  nervous  disorders,  accumulating  experience  shows 
that  typical  pictures,  so-called,  are  the  exception  rather  than  the  rule. 
In  other  words,  there  is  no  one  symptom  that  may  not  be  absent,  and 
very  few  symptoms  that  may  not  be  present  in  disorders  other  than 
tabes. 

Nevertheless  one  can  rely  fairly  well  upon  the  following  grouping 
of  symptoms:  Lancinating  neuralgic  pains,  mostly  in  the  lower  ex- 
tremities, usually  preceding  all  of  the  other  symptoms;  paresthesia 
and  related  sensory  disturbances,  analgesias,  hypo-esthesias,  loss  of 
the  tendon  reflexes  (patellar,  Achilles),  incomplete  or  complete  Argyll- 
Robertson  pupil,  unilateral  or  bilateral  ataxia  in  both  extremities, 
Romberg's  sign,  bladder  disturbances,  hypotonia,  ocular  palsies, 
positive  Wassermann  in  the  blood  and  lymphocytosis  in  the  spinal 
fluid. 

A  host  of  other  symptoms  may  be  present  in  individual  cases,  either 


560  "       SYPHILIS  OF   THE   NERVOUS  SYSTEM 

early  or  late,  but  those  just  mentioned  belong  more  particularly  to 
the  majority  of  the  cases,  and  are  usually  sufficient  to  make  an  early 
diagnosis.  A  description  of  the  individual  symptoms  will  show  some- 
what of  the  proportionate  occurrence,  both  in  point  of  time  and 
frequency. 

Pains. — Severe  lancinating  pains  occur  in  about  90  per  cent,  of  the 
cases,  and  usually  as  an  initial  sign  (60  per  cent.).  The  patients 
complain  in  an  almost  stereotyped  manner  of  having  had  sharp, 
severe,  fugacious  pains  usually  in  the  sciatic  and  crural  distributions, 
which  they  speak  of,  and  unfortunately  are  regarded  even  by  physi- 
cians, as  rheumatic.  These  pains  come  on  in  attacks,  last  a  few  min- 
utes or  more,  several  hours,  a  few  days  and  then  disappear,  to  again 
recur.  They  may  precede  the  development  of  other  symptoms  by  a 
few  months  or  even  many  years  (22  years — Erb) .  The  average  varies 
widely.  They  are  an  indication  of  the  leptomeningitis  or  radiculitis 
which  is  one  of  the  fundamental  results  of  the  syphilitic  virus  or 
products  induced  by  it.  They  are  usually  of  extreme  severity  and 
are  much  dreaded  by  the  patient. 

Whereas  the  distribution  is  predominantly  sciatic  or  crural  and 
radicular  at  first,  the  pains  may  be  widelj^  distributed,  aiid  may  in  fact 
start  in  any  sensory  root,  cranial  or  spinal.  Thus  trigeminal  neu- 
ralgia may  be  an  initial  sign,  or  the  pains  may  affect  the  larynx,  or 
the  stomach,  or  the  heart,  the  bladder,  the  testicles,  the  intestines,  and 
give  rise  to  various  forms  of  crises,  so  characteristic  and  so  much  feared. 
The  pains  may  be  felt  in  the  skin,  or  deep  within. 

Deep,  boring  pains  are  also  present,  usually  later.  Not  infrequently 
the  larger  nerve  trunks  are  somewhat  painful  to  pressure.  This  fact 
may  lead  to  confusion  in  separating  an  alcoholic  or  other  neuritic 
pseudotabes. 

Crises. — These  have  some  relation  to  the  pains  of  tabes,  and  are 
probably  due  to  similar  pathological  alterations,  but  located  in  other 
sensory  and  sympathetic  root  areas.  The  best  known  are  the  gastric 
crises,  noted  as  early  as  1856  by  Gull  and  recognized  by  Charcot 
(1868)  as  belonging  to  the  general  picture  of  tabes.  The  patients  have 
sudden,  violent  gastric  pain,  radiating  in  all  directions,  and  in  the  severe 
attacks  accompanied  by  nausea,  vomiting  and  great  prostration.  Like 
the  pain  attacks,  these  crises  may  last  for  hours,  or  a  few  days  and 
then  disappear  for  weeks  or  months  to  recur  at  irregular  intervals. 
They  disappear  as  rapidly  as  they  come,  and  quite  analogous  to  the 
lancinating  pains,  may  be  early  or  late  symptoms.  Certain  cases  of 
tabes  begin  with  such  crises. 

Similar  crises  affecting  other  internal  organs  have  the  same  etiology 
and  course.  Thus  there  are  intestinal  colics  with  diarrhea,  rectal 
pains  with  tenesmus  and  diarrhea,  vesical  crises  with  strangury,  urethral 
crises,  renal  colic-like  attacks,  testicular  crises,  vulvovaginal  crises, 
laryngeal  and  diaphragmatic  crises  with  cyanosis  and  dyspnea, 
pharyngeal  crises  with  obstinate  hiccough,  bronchial  crises  with  cough. 


TABES 


561 


cardiac  with  angina-like  attacks.  Sneezing  attacks  have  been  described 
as  an  initial  tabetic  sign. 

Sensory  Involvement. — As  a  result  of  the  implication  of  the  men- 
inges of  the  sensory  roots,  alterations  in  the  sensory  functions  take 
place.  The  pathological  process  in  some  is  so  gradual  or  mild  as  not 
to  give  rise  to  pain,  and  in  many,  initial  paresthesias  may  pre- 
cede the  pains,  but  .more  often  the  same  pathological  process  gives 
rise  to  both.    Tingling,  numbness,  crawling  sensations,  flashes  of  hot 


Fig.  282. — -Tabes  involving  the  cauda  equina,  showing  the  radicular  distribution  of 
the  sensory  disturbances.  Tactile  pain  and  thermal  sensibility  were  involved.  The 
patient  first  had  pains  and  sphincter  disturbances.  The  second  dorsal  root  was  also 
slightly  involved,  hence  the  sensory  changes  in  the  arms  (Dejerine). 

and  cold,  slight  benumbing  of  the  tactile  sensibility,  causing  the  sensa- 
tion of  wearing  a  glove,  or  walking  upon  a  textile  are  the  usual  forms. 
They  may  be  be  expected  almost  anywhere  from  the  region  of  the 
trigeminus  through  any  sensory  cervical  nerve  branch  to  the  tips  of 
the  toes,  although  the  ulnar  region  seems  a  site  of  special  predilection. 
Occasionally  they  cause  the  peculiar  girdle-band  sensation,  at  one  time 
considered  so  characteristic  of  tabes.  A  whole  limb  may  be  involved, 
but  under  any  condition  the  tendency  for  the  sensory  disturbances  is 
to  show  a  radicular  distribution  (Dejerine)  (Figs.  282-286). 
36 


562 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


Increasing  sensitiveness  is  also  frequent,  so  that  the  patient  dreads 
the  cold,  or  draughts,  or  sudden  shocks,  or  the  clothing,  not  only  on 
account  of  the  abnormal  sensitiveness,  but  because  these  may  bring 
on  the  severer  pains.  Tactile  anesthesia  follows  this  and  is  a  direct 
resultant  of  the  degenerative  process  in  the  root  area.  These  are  also 
irregularly  distributed. 

Other  sensory  functions  also  become  involved.  The  pain  sense 
may  become  lost  in  irregular  areas.  Retardation  in  the  carrying  of 
pain  impulses  is  present  at  times.  There  may  also  be  numbing  or 
loss  of  heat  and  cold  sensibility.  Bony  sensibility  as  tested  by  the 
tuning-fork  also  may  be  absent  and  shows  irregular  distribution.  In 
general,  deep  sensibility  is  more  profoundly  affected  than  epicritic 
sensibility. 


Figs.  283,  284,  285,  and  286. — Radicular  distribution  of  sensory  disturbances  in 
tabes;  Figs.  283  and  284  represent  the  distribution  of  tactile  anesthesia;  Figs.  285  and 
286  that  of  loss  of  pain  and  temperature  sense  (Dejerine). 

Ataxia. — The  most  prominent  sign  of  the  sensory  involvement  just 
noted  is  seen  in  the  gradually  (sometimes  suddenly)  developing  ataxia. 
The  fibers  conducting  the  impulses  from  the  joints  and  the  muscles 
to  the  chief  organ  for  their  coordination,  the  cerebellum,  are  degenerat- 
ing, and  there  results  an  imperfect  knowledge  of  the  position  of  the 
joints  and  of  the  states  of  muscular  tension  necessary  to  the  proper 
performance  of  motor  functions.  There  results  a  hypotonus  and  sway- 
ing of  the  arms  in  the  finger-nose  test  and  finger-finger  test,  and  of 
the  legs  in  the  knee-heel  test,  and  in  walking.  The  patients  are  par- 
tially or  completely  unaware  of  where  their  limbs  may  be,  and  are 
unable  to  control  the  same,  save  to  a  certain  degree  through  other 
avenues,  namely,  the  eyes.  Hence  with  closed  eyes  all  of  these  signs 
of  ataxia  are  markedly  increased.     With  this  great  uncertainty  of 


TABES  563 

movement  there  is  no  muscular  paralysis,  and  little  loss  of  muscular 
strength,  save  as  the  patient  generally  becomes  weaker. 

Gait  disturbances  are  a  direct  result  of  the  ataxia,  and  are  extremely 
characteristic.  They  were  described  by  Schermer  as  early  as  1819. 
In  the  early  stages  the  patient  notes  a  difficulty  in  going  up  and  down 
stairs,  or  finds  himself  insecure  on  uneven  surfaces.  He  stumbles 
and  at  times  falls.  At  night  he  finds  it  more  difficult  to  get  about,  and 
he  soon  notices  that  he  must  keep  his  eyes  glued  to  his  legs  or  the 
surface  on  which  he  is  walking  if  he  is  to  be  able  to  control  them. 
Later  he  must  walk  with  a  cane,  and  his  legs  are  thrust  somewhat 
wider  apart,  are  thrown  somewhat  irregularly  forward,  and  are  then 
brought  to  the  ground  with  a  sharp  stamp,  the  knee  being  stiffened 
or  even  bent  slightly  backward — overextended — at  times  so  much  so 
as  to  cause  him  to  fall. 

His  step  becomes  quicker  and  more  stamping,  until  he  reaches  a 
point  where,  unless  he  has  trained  himself  to  walk  anew,  he  becomes 
bed-ridden.  His  manner  of  rising  from  a  chair  or  sitting  down  soon 
becomes  very  characteristic.  The  ataxia  in  the  upper  extremities, 
which  is  usually  less  than  that  in  the  lower,  affects  the  patient's  writ- 
ing, the  buttoning  of  the  clothes,  his  dressing,  etc.  His  hands  are  con- 
stantly moving — one  finger  and  then  another  is  raised  or  lowered, 
or  the  wrist  turned.  Oppenheim  has  called  particular  attention  to 
these  spontaneous  movements,  which  closely  resemble  a  static  ataxia, 
and  are  found  throughout  the  body. 

Other  muscles  naturally  may  be  involved  in  the  ataxia,  particularly 
those  [of  the  face,  mouth,  tongue,  larynx,  pharynx  in  which  case 
speech,  singing,  swallowing,  etc.,  are  affected.  Many  patients  die  of  • 
aspiration  pneumonia  through  ataxia  in  the  swallowing  apparatus. 

Ro7nberg's  sign  is  another  result  of  the  loss  of  position  sense.  It 
may  be  an  early  sign,  but  is  more  apt  to  develop  later  in  the  disease. 
Many  patients  without  well-developed  Romberg  are  unable  to 
balance  themselves  on  one  foot,  and  further,  a  mild  Romberg  may 
be  more  readily  demonstrated  by  having  the  patient  bend  slightly 
forward. 

Tendon  Reflexes. — ^Westphal  first  emphasized  the  importance  of  the 
diminution  or  loss  of  the  tendon  reflexes — notably  of  the  knee-jerk 
and  the  Achilles  reflex.  These  belong  among  the  initial  symptoms  in 
the  larger  number  of  cases.  The  knee-jerk  may  be  first  diminished 
on  one  side,  but  demonstrable  by  the  Jendrassik  method,  or  lost,  and 
this  for  years,  perhaps,  before  the  development  of  a  complete  Westphal 
phenomenon.  The  Achilles  jerk  is  lost  in  a  similar  manner  and  not 
infrequently  even  before  the  loss  of  the  knee-jerk  (Babinski  method). 
In  the  upper  extremities  the  diminution  or  loss  of  the  biceps  and 
radial  periosteal  reflexes  are  likewise  early  and  fairly  constant  phenom- 
ena, though  less  marked  than  the  changes  in  the  lower  extremities. 

These  tendon-reflex  changes  are  all  referable  to  the  degenerations 
in  the  root  zones  and  sensory  columns. 


564 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


Cranial  Nerve  Involvement. — Any  one  or  all  of  the  cranial  nerves  may 
be  implicated.     Loss  of  smell  is  rare. 

Pupillary  Reflexes. — Here  a  striking  phenomenon  is  observed. 
Patients  with  tabes — as  with  many  other  syphilitic  affections  of  the 
medullary  or  midbrain  region^ — show  a  diminution  or  loss  of  the  pupil- 
lary light  reflexes,  without  any  loss  of  the  reflex  of  convergence  or  of 
accommodation.  This  is  the  Argyll- Robertson  phenomenon.  It  is 
present  in  over  60  per  cent,  of  the  cases,  and  may  be  present  for 
many  years  without  other  symptoms.  The  pupils  are  apt  to  be  at  first 
irregular  in  size,  and  also  not  infrequently  in  shape.  The  light  reac- 
tion is  at  first  less  prompt — usually  in  one  eye  before  the  other — later 


Fig.  287. — Tabes.     Third  and  sixth  nerve  palsy. 


both  eyes  are  involved.  Myosis  in  marked  degree  is  then  apt  to 
develop.  Loss  of  the  consensual  light  reflex  is  one  of  the  earliest 
signs  of  this  pupillary  change  (Weiler).  The  sympathetic  dilatation  of 
the  pupils  is  also  soon  diminished  or  lost. 

Oj)tic  nerve  changes  are  frequent  and  may  occur  early.  There  is  an 
irregular  gray  atrophy,  with  narrowing  of  the  macular  vessels  and 
gradual  narrowing  of  the  visual  field,  first  for  colors  and  then  blind- 
ness, partial  or  complete,  with  advanced  optic  atrophy. 

Ocular  Palsies. — These  very  frequently  belong  to  the  early  symptom- 
atology and  are  often  fleeting.  Persisting  ocular  palsies  are  more 
often  encountered  in  the  later  stages  of  the  disease.  These  palsies  in 
tabes  occur  quite  irregularly  and  show  themselves  as  atonic  ptoses, 


TABES 


565 


irregular  diplopias — from  fourth  or  sixth  or  third  nerve  affections — 
and  rarely  show  signs  of  a  complete  ophthalmoplegia,  internal  or 
external. 

The  trigeminus  involvement  may 
show  not  only  neuritic  pains  as 
already  noted,  but  also  trophic 
disturbances  of  the  cornea,  herpes, 
irregular  development  of  the  bony 
parts,  etc.  The  motor  root  may 
also  be  partly  paralyzed. 

Facial  palsy  occurs,  but  not 
often. 

The  acoustic  is  occasionally  in- 
volved in  both  cochlear  and  ves- 
tibular branches,  producing  luii- 
lateral  or  bilateral  deafness,  or 
vestibular  nystagmus,  Meniere- 
like  crises,  etc.  (Bonnier's  syn- 
drome). 

The  vagus  and  accessory  nerves 
are  frequently  implicated,  causing 
crises  already  noted,  and  also  slow- 
ing of  the  pulse,  dyspnea,  tachyp- 
nea, etc.  Taste  is  not  infrequently 
lost.    Bulbar  palsies  are  frequent. 

Visceral  Symptoms. — The  bladder  is  usually  involved  at  some  stage 
of  the  disorder.    The  crises  have  been  referred  to.    Weakness  of  the 

bladder  is  the  most  usual  occur- 
rence. Cystitis  usually  develops 
and  is  a  cause  of  general  weak- 
ness. Incontinence  is  not  infre- 
quent. Constipation  is  also  fre- 
quent, but  fecal  incontinence  not 
common.  Loss  or  increase  of 
sexual  desire  is  an  early  sign; 
impotence  is  common;  continuous 
priapism  unusual. 

Trophic  Symptoms.  —  These 
may  involve  any  of  the  tissues 
of  the  body,  but  particularly 
skin  and  bones.  Arthropathies 
are  very  frequent,  usually  occur- 
ring after  the  disorder  is  well 
advanced,  not  infrequently  as  an 
early  symptom.  The  knee-joint 
The  arthropathies  usually  develop 
with  surprising  rapidity — with  edema  and  swelling  but  none  of  the 


Fig.   2S8. — Tabes.     Third  nerve  palsj'. 


Fig.  289. — Tabetic  arthropathy  of  the  rib, 


is  the  joint  of  special  predilection. 


566 


SYPHILIS  OF  THE  NERVOUS  SYSTEM 


usual  signs  of  a  rheumatic  joint;  then  new  bone  formations  take  place, 
with  or  without  subluxation.  Almost  any  joint  may  be  affected,  even 
the  jaw. 


Fig.  290. — Tabetic  arthropathy  of  ribs. 


Fig.  291. — Tabetic  arthropathy  in  the  wrist. 


TABES 


567 


Fragility  of  the  bones  is  a  further  complication.    Perforating  ulcers 
of  the  feet  constitute  another  trophic  disorder. 


Fig.  292. — Tabetic  arthropathy  with  destruction  of  the  joint  and  hypotonus. 


Fig.  293.— Charcot  joint. 

Muscular  Atrophies. — An  initial  neuritis  may  give  rise  to  an  early 
muscular  atrophy,  but  atrophies  are  not  common.' 

1  See  Lapinsky,  Arch.  f.  Psy..  40-42. 


568  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

Blood  Serum  and  Cerebrospinal  Fluid. — The  Wassermann  reaction 
of  the  blood  serum  is  positive  in  most  of  the  cases  of  tabes.  The  cere- 
brospinal [fluid  Jis  positive  in  from  60  to  80  per  cent.,  and  Plant  is  of 
the  opinion  that  it  will  be  found  to  be  more  often  so  with  improved 
technique.  Nonne  and  Hauptmann,  by  using  larger  quantities  of 
serum,  have  established  this.  It  seems  to  be  less  often  positive  than  in 
paresis — which,  in  view  of  the  opinion  held  concerning  the  identity 
of  the  two  disorders  is  food  for  reflection  regarding  the  local  reaction 
of  the  brain  and  cord  proper. 

There  is  a  marked  lymphocytosis  of  the  fluid,  which  is  usually  found 
in  the  early  stages  of  the  disorder,  and  is  an  index  of  the  meningitic 
syphilosis  usually  found. 

Course  and  Variation. — Tabes  is  essentially  a  chronic  disorder,  usually 
mildly  progressive,  developing  fully  after  many  years.  In  a  small 
number  of  cases  it  runs  an  acute  course,  causing  death  within  a  few 
years.    In  others  it  is  stationary  and  in  this  sense  gets  well. 

Duchenne  (1858-1859),  in  his  memorable  description,  made  a  divi- 
sion into  three  stages,  which  have  been  somewhat  modified.  The 
periods  usually  considered  at  the  present  time  are  as  follows:  (1) 
Prodromal  or  preataxic  stage;  (2)  ataxic  period;  (3)  paralytic  period. 

Such  a  division  is  of  arbitrary  value  only;  no  two  cases  are  exactly 
alike.  The  preataxic  stage  may  last  many  years,  or  there  may  be  none, 
paralysis  and  ataxia  developing  with  extreme  rapidity.  The  symptoms 
are  so  many,  and  the  times  of  their  appearance  so  variable,  that  a 
so-called  typical  course  is  the  exception  rather  than  the  rule,  yet  a 
not  unusual  course  is  one  extending  over  about  ten  years,  with  two 
to  three  years  of  pains,  with  or  without  crises,  then  the  gradual  devel- 
opment of  the  loss  of  knee-jerks,  Argyll-Robertson  pupils,  gradually 
increasing  difficulty  in  walking,  worse  in  the  dark,  Romberg — then 
the  patients  are  confined  to  bed,  and  then  the  stage  of  paralysis  and 
atrophies. 

Death  results  from  the  disease  itself,  or  from  complicating  disorder 
— bulbar  accidents  causing  pneumonia,  laryngeal  choking,  cardiac 
syncopes,  kidney  complications,  very  often;  intercurrent  disease 
such  as  tuberculosis,  in  large  part,  pneumonia,  typhoid,  and  erysipelas 
in  smaller  percentages. 

Prognosis  is  always  most  sinister.  Stationary  cases  are  known,  but 
a  question  concerning  diagnosis  may  be  raised  respecting  the  cases 
in  the  older  literature.  With  the  newer  objective  Wassermann  and 
cytological  symptoms  it  is  to  be  seen  whether  such  stationary  cases 
exist.  Since  such  are  found  as  paretics,  it  is  not  improbable  that  the 
same  will  hold  true  for  tabes. 

The  cases  with  severe  bladder  complications  usually  do  badly. 
The  duration  of  life  has  varied  from  six  months  to  thirty  and  more 
years.    The  general  average  runs  between  ten  to  fifteen  years. 

Forms. — Certain  types  are  worthy  of  special  mention  as  forms, 
these  are: 


TABES— DIAGNOSIS  569 

(1)  Juvenile  Tabes,  like  juvenile  paresis,  occurs  in  children  from 
five  to  ten  j^ears  of  age,  or  in  young  adults  apparently  up  to  about 
twenty-five  years.  The  higher  age  incidence  is  rare.  The  pupillary 
signs  are  early,  and  the  bladder  is  soon  involved.  Ataxia  and  paraly- 
sis then  develop.  The  objective  serological  and  cytological  changes  are 
usually  positive — the  number  of  cases  examined  is  as  yet  too  small  to 
permit  wide  generalizations,  but  syphilis  of  the  parents  is  an  essential. 

(2)  Late  Tabes. — The  appearance  of  the  disorder  after  the  age  of 
fifty  is  rare,  yet  cases  are  recorded  in  which  the  initial  signs  have 
come  on  as  late  as  seventy  years.  One  case  is  on  record  of  infection 
at  twenty,  tabes  at  fifty-nine.  After  all  it  is  mostly  a  question  of  when 
infection  takes  place — since  cases  of  infection  at  eighty  or  over  are 
known,  it  is  practically  impossible  to  determine  the  tabes  age  limit. 
No  noteworthy  symptomatic  variations  are  known  in  late  tabes. 

(3)  Rapid  Tabes. — Here  the  disease  advances  very  rapidly,  render- 
ing the  patient  incapacitated  in  a  few  months,  with  death  as  a  result 
of  the  paralyses.  The  paralyses  and  atrophies  occupy  the  foreground 
in  the  picture,  the  ataxias  being  less  prominent.  The  pupillary  signs 
are  present.    Death  may  take  place  within  six  months. 

(4)  Slow  Cases. — These  are  the  more  usual  cases  already  described. 

(5)  Stationary  or  Benign  Cases. — A  particular  type  in  which  blind- 
ness comes  on  early  and  which  follows  a  benign  course  was  first 
described  in  1881  by  Benedikt.  Dejerine  and  Martin  {These  de  Berne, 
1890)  called  attention  to  the  fact  that  it  is  rare  to  find  a  case  of  tabes 
beginning  with  blindness  that  advances  to  the  second  stage. 

Furthermore,  amaurotic  tabetic  cases  seem  to  have  fewer  pains. 
The  pathological  features  are  the  same  as  in  other  cases,  save  as  to 
extension,  and  the  explanation  of  this  variation  is  difficult  to  find. 
A  certain  diminution  in  the  general  symptoms  in  amaurotic  tabes  has 
been  observed. 

Diagnosis. — Little  difficulty  exists  after  the  development  of  the 
Argyll-Robertson  pupil,  lost  knee-jerks,  ataxias,  and  Romberg. 
Practically  the  only  differential  at  this  stage  is  a  polyneuritic  process, 
principally  of  alcoholic  origin.  Here  the  pupillary  disturbances  are 
less  in  evidence,  but  can  occur,  and  if  present  there  are  usually  more 
grave  cerebral  symptoms  and  a  diagnosis  of  taboparesis  is  more  in 
question.  The  serological  and  cytological  results  determines  a  diagnosis 
almost  at  once.  Still  the  most  difficult  cases  to  differentiate  are  those 
of  alcoholism  complicated  with  syphilis.  Where  the  alcoholism  causes 
a  pseudotabetic  picture,  and  the  syphilis  gives  its  serological  and 
cytological  findings  with  slight  meningitic  affection  as  its  only  spinal 
or  cerebral  concomitant.  These  cases  are  diagnosed  only  with  the 
greatest  of  difficulty. 

Other  toxic  pseudotabes  offer  no  difficulties — such  as  those  due  to 
ergot,  diabetes,  lead,  pernicious  anemia,  and  the  infectious  toxemias. 
Here  the  pupillary  signs  are  absent  for  the  most  part,  and  the  serum 
and  spinal  fluid  normal. 


570  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

Certain  cerebellar  atrophies  and  new  growths  cause  symptoms  closely 
resembling  those  of  tabes,  but  the  gait  is  more  widely  swaying,  the 
individual  movements  have  less  ataxia,  the  absent  knee-jerks,  and 
Argyll-Robertson  are  wanting,  and  there  are,  moreover,  to  be  found 
the  definite  signs  of  cerebellar  involvement  in  the  nystagmus  and  its 
alterations  by  the  Barany  vestibular  tests.  Normal  serum  or  cord 
findings  are  to  be  expected. 

Multiple  sclerosis  can  occasionally  cause  a  tabetic  syndrome  if  a 
patch  should  involve  the  sensory  neurons,  but  here  the  other  symptoms, 
the  nystagmus,  the  signs  of  spasticity,  masked  by  the  hypotonus, 
Babinski  phenomena,  etc.,  should  afi^ord  the  clue.  Rare  sclerotic 
patches  in  the  pons  and  midbrain  regions  have  caused  unilateral 
Argyll-Robertson. 

Syringomyelia  occasionally  develops  with  an  initial  tabetic  picture, 
but  soon  the  classical  dissociation  shows  itself,  and  permits  a  diagnosis. 
The  blood  and  spinal  fluid  findings  are  also  to  be  reckoned  with. 

Hysteria  as  an  astasia  abasia  occasionally  causes  difiiculty,  but 
here  careful  examination  can  exclude  the  entire  tabetic  symptom- 
atology. 

Pseudotabetic  neuritides  and  eye  signs,  i.  e.,  irregular  pupils,  gastric 
crises,  may  occur  in  masked  myxedema  (hypothyroidism)  of  later 
years. 

Pathology  and  Pathogenesis. — Both  Ollivier  d'Angiers  and  Cruveilhier 
gave  descriptions  of  the  general  gross  anatomy  and  both  recognized 
the  sclerosis  of  the  posterior  columns.  Todd  associated  the  sclerosis 
with  the  ataxia.  Burdon  and  Luys  (1861)  called  attention  to  the  rela- 
tion of  the  sclerosis  of  the  posterior  roots,  and  the  posterior  column 
sclerosis,  since  which  time  the  development  of  knowledge  concerning 
the  pathological  processes  in  tabes  have  been  most  actively  studied, 
although  be  it  said,  without  yet  arriving  at  general  uniformity. 

Seen  with  the  naked  eye,  the  cord  is  usually  markedly  atrophied, 
hence  the  origin  of  the  old  term  consumption  (tabes);  the  posterior 
roots  are  atrophied,  sometimes  more  marked  in  one  region  than 
another.  The  diminution  in  volume  of  the  cord  also  varies  in  places, 
being  more  pronounced,  as  a  rule,  in  the  dorsal  and  sacrolumbar  region 
than  the  cervical,  and,  as  Cruveilhier  noted,  the  atrophy  preponderates 
markedly  in  the  posterior  columns.  The  pia  and  arachnoid  are  some- 
what swollen  but  translucent  with  slight  opalescences  or  cloudiness. 
Meningeal  involvement  is  very  frequent;  a  fact  brought  out  more  in 
recent  years,  and  in  strict  conformity  with  the  findings  of  the  cellular 
contents  in  the  cerebrospinal  fluid. 

Throughout  the  entire  length  of  the  cord  one  finds  a  graying  dis- 
coloration, the  margins  are  slightly  sunken  below  the  normal  level, 
and  the  discolored  areas  are  harder  to  the  touch.  The  atrophy  seems 
to  cease  with  the  sensory  medullary  nuclei — save  in  those  cases  where 
the  cranial  nerves  are  markedly  affected  when  irregular  atrophies  are 
encountered  in  the  upper  sensory  neurones. 


TABES— PATHOLOGY 


571 


Histulogically  the  picture  is  fairly  uniform.  There  is  a  mild  inflam- 
matoF}^  thickening  of  the  pia  and  arachnoid  with  lymphocyte  and 
plasma-cell  infiltrates.  This  leptomeningitis  varies  considerably  in 
its  localization,  and  in  its  intensity,  and  the  vessels  are  not  infre- 
quently involved.  The  whole  process  closely  approaches  that  of  a 
syphilitic  meningitis,  but  is  less  intense,  and  is  not  accompanied  by 
the  presence  of  spirocheta. 

The  posterior  roots  are  irregularly  atrophied;  the  sacral  and  lumbar 
roots  may  show  not  a  single  unchanged  fiber,  whereas  the  cervical 
roots  are  less  involved,  save  in  the  case  of  cervical  tabes,  where  the 
reverse  holds  true. 

The  posterior  columns  show  the  most  uniform  lesions.  These  are 
greatly  diminished  in  volume.  There  is  a  gradual  degeneration,  often, 
however,  more  intense  on  one  side  than  the  other.     The  atrophy  is 


Fig.  294. — Tabetic  changes  in  cord  in  paresis. 


not  universal,  for  many  fibers  are  intact.  These  are  usually  col- 
laterals, originating  within  the  cord  itself.  The  direct  fibers  in  the 
columns  of  Goll  and  Burdach,  from  the  degenerated  posterior  roots, 
are  those  chiefly  involved.  A  great  deal  of  variability  exists  as  to  the 
respective  distribution  of  the  atrophic  degenerated  fibers;  but  such 
bear  a  direct  proportion  to  the  distribution  of  the  degenerated  pos- 
terior roots.  Certain  portions  of  the  cord  escape,  due  to  anatomical 
reasons;  these  are  more  particularly  the  commissural  zones,  Flechsig's 
centrum  ovale,  Gombault  and  Phillippe's  triangle  in  the  lumbosacral 
region;  the  cornucommissural  zones,  Schultze's  comma  tract  and  the 
fascicles  of  Hoche.     (See  Plate  X.) 

As  a  result  of  the  disappearance  of  the  fibers  a  secondary  neuroglia 
infiltration  takes  place.  This  consists  of  glia  with  fine  prolongations, 
and  also  spider  cells.  In  the  cases  of  taboparesis  one  finds  lympho- 
cytes and  plasma  cells  within  the  cord  substance.     With  a  limited 


572 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


tabetic  process  they  are  not  usually  encountered.^  Many  ganglion 
cells  of  the  posterior  horns  show  degenerative  changes.  They  are 
diminished  in  size,  there  is  definite  chromatophilia,  or  vacuolization, 
and  other  signs  of  degeneration.  The  cells  of  the  column  of  Clark 
are  diseased  only  to  a  slight  extent. 

In  tabes  with  cranial  nerve  signs  the  degeneration  can  be  traced  in 
the  medulla  and  pons  with  secondary  atrophy  of  the  sensory  nuclei. 
Atrophy  of  the  optic  nerve  is  not  infrequent. 


Diira 


AnteriorRoot 


Arachyioid, 


Posterior  Root. 


AnteriorBoot 

lYeaj'iiis 


Gan^h'o7i 


RootZojie 
i/ivolved  mlades. 

Poster/or  Root 

JVeurifis. 


Fig.  295. — Nageotte's_scheme  representing  the  location  of  the^chief  lesion  of  tabes  in 

the  posterior  root  zone. 

Jendrassik,  Schaffer,  Epstein  and  Krauss  have  also  shown  that 
the  brain  cortex  is  not  uninvolved  even  in  typical  tabes.  There  is 
a  diminution  in  the  nerve  fibers,  and  alterations  in  the  vessels  and  pia 
— there  may  be  lymphocyte  and  plasma-cell  infiltration  even  in  the 
absence  of  pronounced  mental  symptoms.  Cerebellar  degenerations 
are  also  known.  The  anterior  horn  cells  do  not  always  escape.  In 
the  patients  with  pronounced  atrophy  many  motor  cells  of  the  anterior 
horns  are  found  degenerated.  Similar  changes  are  known  where  there 
are  bulbar,  laryngeal  or  other  cranial  nerve  palsies.    The  cerebrospinal 


'  Alzheimer,  N.  Arb.,  i,  14. 


SYPHILITIC  MENINGOMYELITIS  573 

fluid  also  shows  the  effects  of  the  mild  inflammatory  process  by  its 
increased  lymphocytosis;  a  count  of  over  10  cells  to  the  c.cm.  (Fuchs 
and  Rosenthal  chamber)  is  to  be  regarded  as  positive,  50  to  100  are 
not  unusual.     Plasma  cells  are  also  to  be  found. 

Serologically  a  positive  Wassermann  is  to  be  expected,  although 
here  the  number  of  positive  results  would  seem  to  fall  below  that 
obtained  in  paresis.    Possibly  paresis  indicates  a  more  acute  process. 

Nonne  phase  I  reaction  is  also  frequently  positive. 

The  posterior  ganglia  are  also  affected  in  many  cases,  although 
not  uniformly.  There  is  atrophy  and  destruction  of  the  cells,  and 
proliferative  inflammatory  exudates  of  the  capsule.  The  changes  are 
not  sufficiently  constant  to  permit  one  to  assume  that  the  primary 
part  of  the  disorder  is  located  in  the  posterior  ganglia. 

The  peripheral  nerves  are  also  frequently  found  degenerated; 
Nonne  says  constantly;  thus  showing  the  complete  degeneration  of 
the  sensory  neuron,  central  as  well  as  peripheral. 

Sympathetic  System. — This  shares  in  the  general  destruction,  and 
shows  particularly  in  the  regions  involved  by  gastric  or  other  visceral 
crises. 

Pathogenesis. — Unanimity  of  opinion  has  not  yet  been  reached. 
The  various  hypotheses  evoked  have  upheld  vascular  (meningeal), 
medullary,  radicular  and  the  neuritic  theories,  not  to  mention  the 
idea  of  a  primary  system  intoxication.  The  general  tendenc}^  is  to 
regard  as  jundamental  a  primary  syphilis  involving  particularly  the 
posterior  roots — a  modified  syphilitic  radiculitis  as  the  most  constant 
feature  in  the  pathogenesis.  The  process  is  one  of  chronic  specific 
poisoning  in  which  there  is  (1)  an  involvement  of  the  posterior  radic- 
ular fibers,  and  of  the  peripheral  nerves,  (2)  an  extension  to  the 
motor  nerve  system,  and  (3)  to  the  sympathetic  fibers. 

Concerning  the  nature  of  this  poison  little  is  known.  It  shows 
certain  analogies  to  an  anaphylactic  substance,  but  not  sufficient 
enquiries  have  been  pushed  far  enough  along  these  lines  to  permit  a 
working  hypothesis.^ 

SYPHILITIC  MENINGOMYELITIS. 

Nonne  has  devoted  a  large  portion  of  his  noted  monograph  to  a 
consideration  of  the  lesions  of  syphilis  of  the  spinal  cord  and  its  mem- 
branes. This  is  a  general  indication  of  its  extreme  frequency,  yet 
most  patients  showing  syphilitic  lesions  of  the  cord  also  show  signs 
in  the  brain  or  its  meninges.  They  are  nearly  all  examples  of  cere- 
brospinal syphilis.  For  practical  purposes,  however,  it  has  been  found 
of  value  to  arbitrarily  divide  this  large  conglomeration  and  discuss  it 
under  two  captions:  cerebral  syphilis,  and  spinal  syphilis  or  meningo- 
myelitis.     This  means  simply  that  we  are  dealing  with  cerebrospinal 

1  See  Head,  Brain,  1913. 


574 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


syphilis  with  predominant  cerebral  and  minor  spinal  symptoms  on 
the  one  hand,  and  with  predominant  spinal  and  nerve  root,  with  less 
prominent  cerebral  signs  on  the  other.  It  again  seems  advisable  to 
accentuate  the  purely  arbitrary  nature  of  all  such  classifications. 

Symptoms. — In  considering  meningomyelitis  as  a  unit,  we  find 
cause  for  further  emphasis  upon  separable  symptom  groups.  Within 
this  conglomeration  again  clinical  neurology  shows  four  fairly  clear 
tendencies : 

1.  Syndromes  due  to  pronounced  meningeal  implication. 

2.  Sjmdromes  due  to  root  and  cauda  equina  disease.  Radiculitis 
and  neuritis. 

3.  Myelitic  syndromes  due  to  indiscriminate  transverse  disease. 


Fig.  296.— Meningomyelitis.     Radiculitis.     Degeneration  of  Burdach's  columns. 

4.  Syndromes  of  less  extensive  transverse  lesions  and  fiber  tract 
isolation.    System  syndromes. 

A  combination  of  all  would  make  a  complete  meningomyelitic  syn- 
drome. This  is  a  not  unusual  picture  in  a  rapidly  developing  case;  in 
its  more  chronic  course  the  emphasis  seems  to  be  laid  upon  one  or 
another  of  the  just-mentioned  groupings. 

These  are  characteristic  syndromes  of  the  early  and  secondary 
stages  of  syphilis.  The  syndromes  may  develop  within  a  few  months 
after  infection,  or  only  come  on  after  many  years.  In  the  former 
case  the  acute  myelitic  changes  are  frequent,  also  root  lesions  (many 
neuralgias,  sciatica,  etc.).  The  later  developing  cases  show  more  the 
systemic  lesions  and  gradually  advancing  meningopathies  (later 
secondary  meningitis)  with  compression  (spastic)  phenomena. 


SYPHILITIC  MENINGOMYELITIS 


575 


In  all  one  expects  to  obtain  a  positive  Wassermann;  cerebrospinal 
fluid  Wassermann  is  negative,  save  with  large  quantities  of  fluid; 
lymphocytosis  is  frequent — often  the  cell  count  being  very  high, 
always  indicating  the  grade  of  meningeal  involvement.  The  lympho- 
cytes are  not  found  before  the  stage  of  roseola;  are  abundant  in  the 
active  secondary  stages,  and  less  frequent  in  the  tertiary  stages  of  a 
meningomyelitis.     The  protein  content  varies  considerably. 

1.  Meningeal  Syndromes. — Severe  pains  are  signs  of  meningeal 
involvement.  They  shoot  across  the  shoulder-blade,  in  the  neck, 
across  the  hips,  dart  down  the  arms  or  legs  and  cause  a  stiffness  of 
the  neck,  the  shoulders  and  the  thighs.  The  spinal  column  is  usually 
sensitive  to  pressure,  and  to  percussion,  and  local  intensities  may 
show  both  these  signs,  and  also  the  peripheral  signs  of  a  definite  zone 
localization. 


Fig.  297. — Radicular  distribution  of  sensory  loss  in  tabes,  particularly  to  tactile 
pain  and  thermal  sensibility.     (Dejerine.) 

Pain  is  frequently  preceded  by  paresthesise,  like  the  crawling  of 
ants,  numbness,  and  coldness.  There  is  a  gradual  increase  in  the 
reflex  excitability  of  the  cord,  due  to  pressure  and  evidenced  by 
increased  knee-jerks,  possible  Babinski  sign,  Oppenheim  or  Chaddock 
signs.  When  pressure  is  exerted  in  the  sacral  segments  bladder  and 
rectal  disturbances  are  frequent. 

With  chronic  meningeal  thickening  these  pressure  symptoms 
increase  markedly,  and  spastic  paretic  phenomena  augment,  especially 
when  gummas  add  their  special  pressures.  Gummata  may  give  rise 
to  a  "cord  tumor"  syndrome. 

2.  Root  Syndromes. — Here  pain  is  frequent  and  neuritic  atrophies 
appear.  Sensory  losses  of  a  root  distribution  are  in  evidence.  Many 
show  the  characteristic  reversal  of  epicritic  touch  loss  being  less  exten- 


576  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

sive  than  protopathic  pain  loss  as  pointed  out  by  Head  as  pathogno- 
monic of  radicular  lesions. 

The  atrophy  of  the  muscles  also  follows  the  radicular  distribution. 

Many  obstinate  neuralgias  are  due  to  syphilitic  radicular  disease. 
Possibly  one-half  of  the  sciaticas  are  of  this  nature.  Dejerine  has 
put  them  as  high  as  80  per  cent,  in  Paris.  A  very  large  propor- 
tion of  the  brachial  neuralgias,  so  long  looked  upon  as  rheumatic  or 
gouty,  or  what  not,  are  due  to  a  syphilitic,  root  meningitis. 

Neuritic  muscular  atrophy,  from  pressure  on  the  anterior  roots, 
is  further  complicated  by  pressure  on  the  anterior  horns  by  the 
thickened  meninges.  Thus  very  anomalous  atrophies  result.  When 
occurring  in  the  eighth  cervical  and  first  dorsal  region  one  obtains 
classical  Klumpke  paralysis  with  dilatation  of  the  pupil,  and  nar- 
rowing of  the  palpebral  jBssure  of  the  affected  side.  Lower  localizations 
result  in  intercostal  palsies,  back  muscle  atrophies,  hip  girdle,  thigh 
or  Cauda  lesions.  In  this  latter  situation  striking  dissociations  are 
obtained,  as  in  the  upper  arm  region,  and  radicular  sensory  disturb- 
ances and  lost  reflexes  are  the  rule  with  atrophies. 

3.  Myelitic  Syndromes. — These  indicate  the  complete  involvement 
of  the  cord,  and  also  point  to  intraspinal  vascular  disease,  rather 
than  to  a  meningeal  lesion.  Complete  flaccid  palsy  is  the  usual  result. 
This  is  combined  with  sensory  loss  as  well.  The  completeness  of  the 
sensory  loss  varies  considerably,  and  indicates  the  severity  of  the 
lesion.  Absence  of  a  lymphocytosis  points  to  a  purely  vascular,  and 
usually  focal  lesion  within  the  cord.  The  bladder  and  rectal  functions 
are  implicated  as  well. 

In  the  regressive  stage  an  increase  in  spasticity  marks  the  sub- 
sidence of  the  inflammatory  reaction,  and  many  anomalous  syndrome 
mixtures  result.  This  phase  of  meningomyelitis  offers  abundant 
opportunity  for  very  heterogenous  syndromes.  A  Brown-Sequard 
complex,  poliomyelitis,  tabetic  syndrome  with  atrophy,  amyotrophic 
lateral  sclerosis  syndrome — these  are  but  a  few  of  the  possible 
combinations. 

4.  System  Syndromes. — These  occur  not  so  much  as  residuals  of  the 
previous  myelitic  changes,  nor  as  due  to  meningeal  compressions,  but 
represent  disease  in  or  about  the  long  motor  tracts,  tractus  cortico- 
spinalis,  especially.  They  give  rise  to  the  forms  of  primary  lateral 
sclerosis  (Erb),  some  combined  scleroses,  and  particularly  to  clinical 
pictures  closely  resembling  multiple  sclerosis.  Combined  disease  of 
the  posterior  and  lateral  columns  is  quite  apt  to  be  syphilitic. 

Special  localization  of  one  or  more  of  these  forms  of  meningeal 
syphilis  gives  rise  to  the  special  forms  of  hypertrophic  cervical  pachy- 
meningitis, which  have  been  described  by  Joffroy  and  Charcot,  and  to 
Kahler's  disease. 

The  anterior  horns  may  be  predominantly  involved  usually,  however, 
as  a  result  of  a  transverse  myelitis  (Nonne)  giving  the  picture  of  an 
anterior  poliomyelitis. 


CONGENITAL  OR  HEREDITARY  SYPHILIS 


577 


In  hypertrophic  cervical  pachymeningitis  one  finds  an  enormous 
thickening  of  the  meninges,  with  or  without  gummata,  and  located 
in  the  cervical  region.  Here  root  and  compression  symptoms  are 
present.  Pains  in  the  neck  and  shoulder,  stiffness  of  the  cervical 
spine,  shooting  pains  down  the  arms.  Sensory  loss  may  then  show, 
particularly  to  pin  prick,  with  relatively  intact  sensibility  to  cotton- 
wool. The  ulnar  and  median  are  particularly  implicated.  Fibrillary 
contraction  of  the  muscles,  atrophy  and  loss  of  electrical  excitability 
occur.  The  special  type  of  deformity  known  as  preacher-hand  is  one 
of  the  frequent  expressions  of  the  involvement  of  the  brachial  plexus 
in  the  cervical  meningitis. 


Fig.  298. — Pachymeningitis  hypertrophica  cervicalis. 


CONGENITAL  OR  HEREDITARY  SYPHILIS. 

Effect  of  Hereditary  Syphilis.  Serological  studies  have  thrown  much 
light  on  the  question  of  the  mode  of  transmission.  This  cannot  be 
entered  upon  here.  These  studies  as  particularly  carried  out  by  Plant, 
Mott,  and  others  have  shown  the  enormous  importance  of  transmitted 
syphilis  in  the  pernicious  effects  upon  the  nervous  system.  Linser, 
moreover,  has  shown  that  two-thirds  of  the  children  of  syphilitic 
parents  show  a  positive  Wassermann  reaction,  although  much  fewer 
show  signs  of  congenital  syphilis. 

It  may  be  recalled  that  Fournier  stated  the  proportion  as  high  as 
98  per  cent.,  and  that  68.5  per  cent,  of  the  children  died.  This  does 
37 


578 


SYPHILIS  OF   THE  NERVOUS  SYSTEM 


not  include  the  aborted  offspring.  Should  these  be  reckoned,  one 
could  obtain  a  true  idea  of  the  morbidity  of  syphilis  in  the  young. 
Hochsinger  reports  an  interesting  group  of  cases  in  this  connection. 
In  72  families  there  was  paternal  syphilis.  The  mothers  were  not 
syphilitic.  Seventy  mothers  gave  birth  to  307  children — 110  still- 
born, 166  syphilitic,  and  31  healthy.  The  healthy  were  all  the  last 
born  save  in  four  instances. 


Fig.  299. — Congenital  syphilis:  Juvenile  paresis,  at  first  considered  as  "imbecile." 

(Mott.) 

Of  the  children  of  tabetics,  one  obtains  the  same  story  from  Mott, 
Mendel;  and  others.  Either  no  children,  many  abortions,  many  dead 
children,  few  living,  and  no  one  knows  as  yet  the  fate  of  these.  Cer- 
tainly one-half  are  doomed  to  disease  and  disorder  of  the  nervous 
system. 


Fig.  300.— Congenital  syphilis:  Three  miscarriages;  then  five  children  born  alive 
and  well.  Last  child  snuffles,  Hutchinson  teeth.  Did  well  in  school,  then  deteriorated, 
noisy,  maniacal;  thought  to  be  paretic.  Autopsy  showed  generalized  cerebrospinal 
gummatous  meningitis,  perivascularitis,  and  endarteritis.     (Mott.) 

It  would  appear  that  the  common  effect  of  such  syphilitic  infection 
is  to  reduce  the  resistances  of  the  body  and  its  powers  for  full  develop- 
ment both  in  the  general  body  and  nervous  tissues.  Syphilis  dimin- 
ishes the  vital  energy  of  the  germ  plasm  prior  to  conjugation,  and  can 
cause  pathological  variations  in  nervous  structures,  just  as  it  can 
transmit  the  disease  through  the  germ  cells.  The  abundant  studies 
on  alcohol  and  its  influence  on  the  germ  cell  affords  an  analogy  in 
understanding  how  this  takes  place  with  another  type  of  toxemia. 


CONGENITAL  OR  HEREDITARY  SYPHILIS  579 

The  classical  formula  of  Fournier  seems  to  hold — abortion,  dead 
child,  early  death,  living,  healthy  child.  This  is  in  need  of  amend- 
ment; it  is  worse.  The  formula  reads:  Complete  sterility,  miscar- 
riage, abortion,  stillbirths,  children  dying  in  infancy  or  convulsions, 
marasmus,  meningitis,  hydrocephalus.  Then  follow  children  who  are 
comparatively  healthy,  but  who  in  later  life  develop  late  hereditary 
syphilis. 

A  study  by  Hochsinger  (1911)  says  that  of  208  children  of  syphilitic 
parents  who  had  been  under  observation  over  four  years,  89,  or  43 
per  cent.,  had  some  disease  of  the  nervous  system.  Of  these  there 
were  9  cases  of  hydrocephalus,  2  of  Little's  syndrome,  6  epileptics,  2 


Fig.  301. — Brain  of  a  congenital  syphilitic  idiotic  child. 

paresis,  1  tabes,  6  Argyll-Robertson  pupil,  36  extremely  neurotic, 
5  hysterical,  14  chronic  headaches,  10  imbeciles.  This  is  in  strange 
contrast  to  the  statements  of  Johnathan  Hutchinson,  who,  but  a  few 
3^ears  ago,  taught  that  nervous  syphilis  was  negligible. 

It  has  been  abundantly  shown  that  nearly  every  form  of  adult 
syphilis  of  the  nervous  system  can  be  encountered  in  hereditary 
syphilis,  and,  as  Mott  well  says,  if  congenital  sj^philis  were  not  so 
fatal  to  infant  life  the  number  of  people  suffering  from  brain  disease 
and  syphilis  would  be  appalling.  It  would  therefore  be  of  little 
service  to  repeat  what  has  already  been  written  regarding  nervous 
syphilis  of  adults  as  it  appears  in  children,  and  the  present  discussion 


580  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

will  be  limited  to  a  consideration  of  such  forms  of  juvenile  nervous 
syphilis  as  are  present  only  in  children.  These  are,  particularly, 
certain  forms  of  feeble-mindedness,  of  hydrocephalus,  ependymitis, 
Freidreich's  ataxia,  primary  optic  atrophy,  and  encephalitides  or 
encephalomalacias,  leading  to  various  hemiplegic  syndromes,  often 
loosely  grouped  together  as  Little's  disease.  Juvenile  paresis  and 
juvenile  tabes  are  among  the  commonest  congenital  disorders  of  later 
infancy  ot  adolescence. 

Congenital  syphilis  of  the  nervous  system  may  show  itself  before 
or  at  birth;  it  may  develop  in  earliest  infancy  or  in  adolescence;  it 
may  develop  as  late  as  twenty,  or  even  forty-two  to  forty-three  years 
(Miiller).  How  long  may  the  virus  remain  latent,  finally  to  develop 
a  definite  syphilitic  reaction?  As  yet  the  answer  will  depend  upon 
the  general  bias  of  the  answerer,  rather  than  upon  empirical  data. 
It  would  appear  from  the  evidence  at  hand  that  no  definite  age  limit 
can  be  put  upon  the  time  when  such  a  latent  factor  becomes  activated 
by  causes  as  yet  unknown.  That  an  activator  of  some  sort  plays 
a  role  in  such  disorders  as  tabes  and  paresis  seems  at  present  a  justifi- 
able hypothesis.  When  a  ncM^born  child,  known  to  have  been  syphil- 
itic biologically,  can  be  followed  throughout  life,  his  serum  reactions 
being  tested  from  year  to  year,  as  is  now  being  done,  then  the  question 
can  be  finall}^  decided. 

In  this  connection  a  line  may  be  devoted  to  the  subject  of  con- 
genital syphilis  "unto  the  third  generation."  While  of  late  years 
doubts  have  been  accumulating  relative  to  this  matter,  certain  positive 
cases  are  being  reported.  The  rationale  of  this  seems  plain  in  view 
of  the  observations  of  recent  years  made  by  Levaditi,  Bab,  and  others, 
that  Treponema  ixdlidum  may  be  found  in  the  ovum,  and  in  an  appar- 
ently resting  stage  similar  to  the  resting  stage  known  for  other 
flagellate  protozoa  closely  allied  to  the  organism  causing  syphilis. 

The  fact  of  the  whole  matter  is  that  opinions  and  statistics  relative 
to  congenital  syphilis  of  the  nervous  system,  supported  by  clinical 
observation  alone,  and  uncontrolled  by  the  available  biological  tests, 
are  insufficient  approximations,  and  very  insecure.  When  relied  upon 
for  negative  purposes  they  are  harmful  to  the  advance  of  thought, 
and  detrimental  to  the  relief  of  sick  humanity. 

Notwithstanding  the  importance,  and  often  the  strikingly  gruesome 
character  of  these  late-appearing  congenital  cases,  the  attention  of 
the  practitioner  should  be  riveted  upon  the  numerically  preponderant 
miscarriages,  stillbirths,  and  early  syphilitic  deaths  if  he  would  get  in 
the  right  attitude  toward  the  therapeutics  of  this  disease,  as  it  affects 
the  nervous  system. 

In  congenital  syphilis,  as  has  been  stated,  one  finds  a  replica  of  what 
has  been  found  in  adult  syphilis.  Pathologically  speaking,  the  lesions 
are  nearly  always  combined.  There  is  a  variable  composite  of  endar- 
teritis, of  leptomeningitis,  of  pachymeningitis,  gummata,  large  and 
small,  localized  or  infiltrating  gummatous  neuritis,  diffuse  degenerative 


CONGENITAL  OR  HEREDITARY  SYPHILIS  581 

changes  in  the  cells  of  the  spinal  cord,  in  the  basal  ganglia,  or  of  the 
cortex.  Thus  the  clinical  pictures  are  apt  to  be  conglomerate,  and 
almost  unanalyzable.  Those  more  accentuated  trends  which  permit 
a  nosological  term  will  be  considered  here. 

Hydrocephalus. — As  a  result  of  congenital  syphilis  this  condition 
has  been  suspected  for  two  hundred  years.  Hasse,  in  1828,  Cruveil- 
hier  in  his  atlas.  Van  Rosen  in  1862,  and  Virchow  reported  definite 
examples  of  it.  It  arises  in  these  congenital  forms  largely  from 
syphilitic  disease  of  the  cerebrospinal  fluid-producing  structures — 
choroid,  ependyma,  or  from  definite  obstructive  factors  in  the  cerebral 
foramina,  gummata,  vascular  swelling  obstructing  the  iter,  etc. 

It  is  a  not  uncommon  sequel  of  congenital  syphilis  and  is  undoubtedly 
more  frequent  than  is  realized.  In  Hochsinger's  series  of  362  cases  of 
congenital  syphilis  34,  or  nearly  10  per  cent,  showed  hydrocephalus. 
In  his  series,  which  affords  a  fairly  average  review  of  the  situation, 
the  hydrocephalus  began  three  to  eleven  months  after  birth;  some- 
times it  was  fetal.  In  11  cases  there  were  no  nervous  symptoms, 
i.  e.,  up  to  the  time  of  reporting.  In  the  others  restlessness,  sleep- 
lessness, chronic  vomiting,  convulsions,  contractures,  nystagmus,  and 
feeble-mindedness  were  the  objective  phenomena. 

The  more  usual  clinical  picture  is  that  of  a  child,  boy  or  girl,  from 
three  to  six  months  of  age,  who  following,  or  not,  an  insignificant 
blow  on  the  head,  or  some  gastro-intestinal  or  bronchial  disturbance, 
develops  within  a  few  days  grave  cerebral  disturbances.  There  is  great 
irritability  and  sleeplessness,  screaming,  and  kicking.  The  head  is 
usually  drawn  back,  the  eyes  and  the  fontanelles  are  apt  to  bulge 
somewhat.  Vomiting  is  frequent,  and  there  are  signs  of  oculomotor 
involvement.  Internal  strabismus  from  paresis  of  the  external  rectus 
is  not  unusual.  It  is  frequently  preceded  by,  or  accompanied  by, 
nystagmus  and  irregular  pupils — often  not  responding  to  light. 

Pain  is  present,  as  the  child  cries  and  struggles,  and  not  infrequently 
the  active  movement  of  the  arms — often  highly  spasmodic  or  convul- 
sive— seem  to  try,  in  a  blind  reflex  sort  of  way,  to  get  at  and  brush 
away  the  source  of  it,  i.  e.,  the  head,  pulling  the  hair,  grasping 
and  rolling  the  head.  Spasticity,  rigidity,  and  other  signs  of  intra- 
cranial pressure  may  at  times  be  demonstrated.  As  a  rule  the  tem- 
perature is  only  slightly,  or  not  at  all  raised,  and  the  minor  signs 
of  an  epidemic  cerebrospinal  meningitis,  i.  e.,  herpes,  temperature, 
flushed  and  spotted  skin,  are  absent.  The  diagnosis  of  all  of  these 
infantile  meningeal  disturbances  is  fraught  with  much  difficulty. 

Feeble-mindedness. — Syphilis  undoubtedly  plays  a  much  larger  role 
in  producing  mental  defectives  than  is  suspected.  The  early  statistics 
are  comparatively  worthless.  They  are  quoted  at  great  length  even 
in  modern  works  on  idiocy,  imbecility,  and  the  like. 

The  more  correct  appreciation  of  this  chapter  on  syphilis  and 
feeble-mindedness  began  with  the  studies  of  Fournier  on  parasyphilis. 
Those  truths,  somewhat  uncontrolled,  were  forced  upon  him  by  his 


582  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

clinical  observations.  The  early  English,  German,  and  American  fig- 
ures varied  from  0.1  (Shuttleworth)  to  17  per  cent.  (Ziehen).  Whereas, 
the  results  following  serological  investigations  start  with  the  higher 
figures,  and  mount  upward,  in  some  cases  as  high  as  40  per  cent. 
The  American  figures  available  (Atwood  and  Clark)  showed  that  20 
per  cent,  of  the  idiots,  imbeciles,  and  morons  at  Randall's  Island, 
New  York,  were  syphilitic. 

Intra-uterine  feeble-mindedness  is  more  or  less  a  contradiction. 
These  children  who  would  date  their  mental  defect  to  disease  going 
on  in  the  uterus  rarely  live.  Plant  expresses  the  opinion  that  feeble- 
mindedness may  be  regarded,  so  far  as  syphilis  is  concerned,  as  the 
result  of  an  extra-uterine  syphilitic  disease  undergone  in  infancy.  In 
some  there  are  signs  of  an  acute  brain  disease.  Some  instances  of 
recovered  hydrocephalus  show  the  signs  of  havoc  in  their  inability  to 
develop  normally.  Many  others  show  no  acute  stage,  but  fail  to 
develop.  Many  recorded  observations  are  available  to  show  the  very 
gradual  development  of  mental  defect,  without  convulsions  or  fever, 
altogether  without  signs  of  organic  disease,  which  arrived  at  a  definite 
termination,  and  which  left  behind  entirely  stationary,  perhaps  even 
improvable,  idiots  or  imbeciles. 

That  type  of  hereditary  syphilitic  child  without  any  tangible  disease, 
formulated  by  Fournier — his  "enfants  arrieres" — who  are  described 
as  unintelligent,  simple,  silly,  limited  children,  always  behind,  not 
infrequently  shows  the  Wassermann  reaction. 

Again,  one  is  convinced  by  the  researches  of  others  that  mental 
defect  in  less-marked  grade,  or  more  properly  speaking,  along  more 
restricted  or  special  lines,  is  allied  with  this  broad  group  on  the  basis 
of  congenital  syphilis.  Thus  Nonne  reports  cases  of  general  irritable 
weakness  of  the  nervous  system.  The  patients  are  highly  excitable, 
are  extremely  nervous,  they  are  very  moody,  suffer  from  headaches, 
irregularities  of  appetite,  sudden  fits  of  passion — not  associated  with 
other  forms  of  epileptiform  analogies — and  for  whom  mercury  and 
the  iodides  worked  wonders. 

Still  another  chapter  has  been  opened  in  this  hereditary  syphilis 
problem  in  its  relation  to  mental  defect.  It  concerns  many  so-called 
psychopathic  children.  These  children  are  bright,  but  they  show 
marked  ethical  defects.  Here  one  can  conceive  of  the  mental  defect 
in  terms  of  limited  cortical  control  to  the  affective  response  of  the 
sexuality,  and  to  the  nutritional  instincts.  These  children  want  and 
take  without  going  around  by  the  circuitous  routes  devised  by 
cultural  standards.  Anatomically  one  can  posit  a  defect  of  cer- 
tain corticocortical  association  areas  on  the  basis  of  the  syphilitic 
poisoning. 

Taking  the  whole  group  of  feeble-mindedness,  it  is  evident  that 
clinically  one  cannot  pick  out  the  hereditary  syphilitic  child  in  all 
instances.  Indeed,  it  should  be  emphasized  that  too  much  weight  is 
given   to   the   anomalies   in   physical   structure — Hutchinson   teeth, 


TREATMENT  583 

saddle  nose,  striae  about  the  mouth,  prominent  veins,  scaphoid  scapula, 
etc. — if  one  rejects  those  who  from  the  hereditosyphilitic  class  fail 
to  show  such  anomalies.  Nor  can  we  recognize  any  certainly  pathog- 
nomonic psychical  anomalies.  A  careful  neurological  examination 
frequently  aids  in  enlarging  the  group — particularly  in  the  study  of 
pupillary  anomalies.  The  cytological  tests  are  of  the  highest  impor- 
tance, and  every  child  born  of  syphilitic  father  or  mother  should  be 
systematically  examined  by  these  cytobiological  methods. 

Treatment. — The  treatment  of  syphilis  of  the  nervous  system  appear- 
ing in  any  of  the  forms  previously  enumerated,  is  often  extremely 
satisfactory.  In  fact,  at  times  one  might  say  the  results  are  often 
too  good,  as  the  speedy  relief  not  infrequently  leads  the  patient  to 
forego  further  treatment,  or  causes  him  to  pursue  his  course  half- 
heartedly. 

For  more  complete  discussion  consult  Modern  Treatment  of  Nervous 
and  Menial  Diseases,  White  and  Jelliffe,  Vol.  II. 

Cerebral  syphilis  for  the  most  part  is  accompanied  by  active  spiro- 
chetes; hence,  the  therapy  is  to  be  directed  against  this  organism. 
For  spirochetal  poisons  we  possess  mercury,  arsenic,  and  iodine. 
Mercury  and  arsenic  are  active,  iodine  is  very  weakly  toxic,  but 
Neisser's  most  recent  studies  tend  to  show  that  it  has  actions  other 
than  that  usually  ascribed  to  it,  namely,  to  promote  the  taking  away 
of  breaking-down  syphilitic  tissue  or  its  product — its  so-called  resorp- 
tion action.  Neisser  shows  that  iodides  in  large  doses  are  toxic  to 
spirochetes  as  well. 

The  treatment  of  nervous  syphilis  then  should  be  an  attempt  to 
follow  out  a  general  antisyphilitic  treatment,  with  special  attention 
to  certain  structural  peculiarities  of  the  nervous  system.  These 
peculiarities  are  of  much  importance.  In  the  first  place,  small  lesions 
in  the  central  nervous  system,  by  impinging  on  important  centers, 
bring  about  disastrous  results,  optic  atrophy,  pontine,  medullary 
hemorrhages,  etc.  The  enormous  importance  of  correct  nervous  and 
mental  functioning  in  the  struggle  for  existence  is  self-evident.  The 
difficulties  in  the  way  of  repair  in  nervous  tissues  are  enormous — at 
times  insuperable. 

Hence,  one's  attack  upon  nervous  syphilis  should  be  prompt  and 
complete.  A  complete  sterilization  is  desirable — and  this  is  often 
extremely  difficult  to  bring  about  in  nervous  tissues. 

It  must  always  be  borne  in  mind  that  cerebral  syphilis  is  often 
present  with  a  negative  Wassermann — this  finding  then  should  not 
deter  one  if  there  are  clinical  signs  of  diagnostic  import.  Not  infre- 
quently active  antisyphilitic  treatment  (salvarsan)  causes  the  appear- 
ance of  a  positive  blood  reaction — how  often  this  occurs  is  not  yet 
known. 

Study  of  the  cerebrospinal  fluid  is  often  a  better  guide  to  the  correct 
appreciation  of  the  situation  as  well  as  a  reflection  of  the  activity  of 
the  process.    Lymphocytosis  is  often  present  months  or  years  before 


584  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

any  definite  nervous  signs.  Lumbar  puncture  is  too  often  neglected 
in  cerebral  syphilis.  Many  authors  state  that  if  the  blood  is  negative 
to  Wassermann  there  is  little  occasion  for  studying  the  cerebrospinal 
fluid.  This  leads  to  bad  results.  Vascular  and  meningovascular 
processes  may  progress  for  years  in  nervous  tissues  without  giving 
rise  to  a  positive  Wassermann.  Here  is  an  occasion  in  which  this 
symptom  fails  to  be  present. 

An  energetic  treatment  should  therefore  be  carried  out  if  there  are 
suggestive  signs  of  nervous  syphilis,  even  should  there  be  a  negative 
Wassermann. 

In  certain  patients  rapid  action  seems  imperative;  in  others  the 
need  for  this  rapidity  is  not  so  much  in  evidence.  Granted  a  knowledge 
of  these  requirements,  the  choice  of  remedies  is  not  as  simple  as  it 
might  seem.  Iodides  given  by  the  mouth  in  doses  of  30  grains,  2 
grams  a  day,  show  evidences  of  activity  in  about  one  week.  Mercury 
by  inunction  shows  results  in  about  five  days,  while  the  newer  arsenical 
preparations  show  reactive  capacities  in  about  forty-eight  to  seventy- 
two  hours.  None  of  these  figures  should  be  accepted  as  final,  so  far 
as  curative  action  is  concerned,  nevertheless  they  are  worth  some- 
thing. It  is  not  apparent  that  iodides  have  a  rapid  toxic  action;  hence, 
in  lesions  which  are  characteristic  of  the  more  florid  aspects  of  spiro- 
chetal growth — basal  meningeal  types  of  acute  onset  particularly — 
they  should  not  be  chosen  in  the  initial  attack. 

Inunction  Method. — For  years  neurologists  have  taught — chiefly 
under  the  influence  of  Erb — that  nervous  syphilis  is  best  attacked  by 
the  inunction  method — combined  with  iodides.  In  those  situations 
in  which  the  time  element  is  of  less  moment  this  attitude  seems  jus- 
tifiable, especially  for  gummatous  types  of  the  disease. 

Oleate  of  Mercury. — Different  possibilities  are  here  presented.  The 
oleate  of  mercury  is  of  value  in  that  it  is  comparatively  cleanly  and 
produces  results  as  rapidly  as  other  mercurial  preparations  applied  to 
the  skin.  A  dram  of  the  10  per  cent,  oleate  is  to  be  used  night  and 
morning  for  four  days.  The  patient  then  takes  a  vapor  bath  and  the 
same  dose  is  used  once  a  day  for  four  days  more.  If  sponginess  and 
soreness  of  the  gums  do  not  appear — with  cleaned  teeth  and  gums — 
the  double  dose  may  be  continued;  otherwise  a  single  dose  should  be 
utilized.  In  using  the  oleate  one  usually  employs  a  small  piece  of 
flannel  in  the  rubbing — the  first  dose  should  be  larger  as  the  flannel 
absorbs  it,  and  the  same  piece  of  flannel  should  be  used  continuously. 

The  oleate  may  irritate  the  skin,  but  as  it  is  absorbed  fairly  well 
from  all  parts  of  the  body  one  can  shift  around  more  readily  with  it 
than  with  other  mercurial  ointments.  Another  object  of  using  the 
oleate  is  on  the  ground  of  secrecy. 

This  line  of  treatment  should  continue  at  least  six  weeks,  after  the 
first  week  10  grains  (0.6  gram)  of  potassium  iodide  t.  i.  d.,  should 
be  administered  during  the  course  of  treatment.  There  is  very  little 
advantage  in  raising  the  amount  of  iodide  above  60  grains  (2  grams) 


TREATMENT  •  585 

a  day.  After  six  or  eight  weeks  the  treatment  should  be  discontinued 
absolutely — to  be  renewed  not  later  than  three  months  after  the  ter- 
mination of  the  last  treatment.  A  third  and  fourth  course  is  advisable, 
even  imperative  if  a  positive  Wassermann  is  present  in  the  blood  or 
lymphocytes  above  10  to  the  c.mm.  are  obtained  from  the  cerebro- 
spinal fluid. 

Ungnentum  Hydrargyri. — Unguentum  Hydrargyri  is  much  used 
and  widely  recommended.  It  has  the  disadvantage  of  being  dirty 
and  of  attracting  attention.  The  latter  may  be  partly  obviated  by 
adding  some  non-stainable  coloring  matter,  or  some  smelling  compound 
such  as  balsam  of  Peru.  Attention  can  thus  be  diverted  from  its 
characteristic  color.  The  ointment  is  used  in  daily  doses  of  from  5j 
(4  grams)  to  5  ij  (8  grams)  best  rubbed  in  in  the  evening  in  a  fairly 
definite  manner,  and  in  places  where  the  skin  is  more  permeable. 
One  uses  the  inner  surface  of  the  arm  and  forearm  for  the  first  rubbings, 
covering  them  with  bandages,  then  the  inside  of  the  groin,  then  the 
popliteal  space,  then  the  abdomen  and  back.  The  fifth  or  sixth  day 
the  patient  omits  his  rubbing — takes  a  Turkish  bath  and  then  starts 
over  the  same  course.    This  course  is  kept  up  for  thirty  doses. 

Other  Details. — ^The  care  of  the  skin  and  of  the  mouth  is  naturally 
to  be  kept  in  mind.  The  blood  Wassermann  should  be  tested  at  the 
end  of  the  period,  and  if  strongly  positive,  or  if  spinal  puncture  shows 
active  lymphocytosis,  or  if  clinical  signs  seem  slow  in  responding,  the 
inunctions  should  be  continued  at  least  two  or  three  weeks  longer. 

Checking  up  by  Wassermann  and  lumbar  puncture  three  to  four 
months  later,  or  any  increase  in  clinical  signs  should  determine  a 
repetition  of  the  treatment  along  identical  or  more  strenuous  lines. 

Iodides,  30  grains  daily,  are  to  be  given  throughout  the  course  of 
the  inunctions.  All  medication  should  cease  at  the  end  of  the  cure, 
unless  there  are  definite  indications  for  its  continuance. 

Other  mercurial  inunction  masses  may  be  used.  Those  of  value 
are  the  hydrargyri  vasenol,  vasogen,  mitin,  resorbin,  which  have 
special  indications  which  may  render  them  particularly  valuable. 

Injection  Treatment. — Injection  treatment  attempts  an  even  more 
rapid  and  energetic  attack  upon  the  spirochete.  Many  battles  have 
been  fought  among  syphilographers  as  to  the  comparative  merits  of 
the  insoluble  or  soluble  salts.  When  so  much  diversity  of  opinion  can 
be  found,  it  usually  indicates  that  the  real  differences  are  usually 
minimal.  Hence,  ease  of  administration,  safety,  painlessness,  etc., 
determine  the  choice  of  the  remedy  in  each  case. 

Calomel,  mercury  salicylate,  and  thymol  acetate  are  among  the 
more  favored  insoluble  salts.  Calomel  has  occupied  a  high  rank 
and  can  be  utilized  in  the  following  forms: 

I^ — Hydrargyri  chloridi  mitis 5.0  gm. 

Sodii  chloridi 5.0  gm. 

Aq.  dest 50.0  gm. 

Mucilagio  arabici 2.5  gm. 

I^ — Hydrargyri  chloridi  mitis. 

01.  sesami,  10  per  cent. 


586  SYPHILIS  OF  THE  NERVOUS  SYSTEM 

Pravaz  syringeful  every  second  or  third  day,  preferably  into  the 
muscles  of  the  thigh  or  back,  for  12  to  15  doses. 

The  hypodermic  use  of  calomel  is  often  accompanied  by  much  pain. 
Abscess  and  necrosis  is  not  uncommon,  and  lung  emboli  may  occur — 
with  care,  however,  calomel  given  by  hypodermic  is  free  from  danger. 

Creams  of  calomel,  devised  by  Lambkin,  have  been  extensively 
used ,  as  they  cause  less  pain  and  give  rise  to  no  complications  if  blood- 
vessels are  avoided.  These  creams  should  be  sterile.  The  formulae 
of  some  in  use  are  as  follows: 

I^ — Calomel 5  gm. 

Creosote 20  gm. 

Camphoric  acid 20  gm. 

Palmitin 100  gm. 

Inject  lOTU  of  this  cream  once  a  week  for  one  month,  to  be  replaced 
by  the  following : 

I^ — Hydrargyri  (metallic) ;      .      .      .      .  10  gm. 

Creosote 20  gm. 

Camphoric  acid 20  gm. 

Palmitin 100  gm. 

This  is  injected  in  doses  of  10  TTL  twice  a  week  for  three  weeks. 

After  six  doses  have  been  given,  no  more  doses  for  two  months. 
Four  injections  of  the  metallic  cream  are  then  given  at  fortnightly 
intervals.  Then  a  rest  for  four  months.  Then  four  injections  as 
before,  and  a  rest  for  six  months.  Then  a  repetition  of  four  fortnightly 
doses — an  interval  of  one  month,  and  a  final  series  of  four  metallic 
cream  doses, 

English  syphilographers  have  found  these  creams  admirable  in 
army  and  navy  work.  They  are  adapted  for  early  stages  better  than 
for  nervous  syphilis,  but  are  worthy  of  more  extended  trial  in  nerve 
syphilis. 

The  use  of  the  insoluble  salts  has  the  advantage  of  a  much  more 
prolonged  action  of  the  mercury.  They  also  have  the  disadvantage — 
all  mercurial  salts  share  in  this,  however — of  irritation  of  the  kidneys. 
If  albumin  is  found  before  the  use  of  mercury  one  should  look  for  a 
syphilitic  albuminuria.  Tuberculosis,  diabetes,  alcoholism,  marked 
cachexia  are  additional  factors  to  be  carefully  dealt  with.  Gastro- 
intestinal disturbances  are  frequent,  but  it  is  extremely  rare  that 
mercury  causes  a  neuritis. 

Thorough  cleansing  of  the  mouth,  and  the  use  of  chlorate  of  potash 
mouth  wash  is  imperative. 

The  soluble  salts  in  use  are  very  numerous.  They  include  the 
sozoiodolate,  bichloride,  lactate,  succinamide,  biniodin,  benzonate,  and 
cyanide.  Fournier  lists  about  30.  They  may  be  injected  within 
the  muscles  or  into  the  skin,  superficially.  All  are  somewhat  painful, 
and  accidents  are  possible.  In  general  the  dosage  is  from  |  to  |  of 
a  grain.    The  injections  are  given  twice  or  three  times  a  week. 


TREATMENT  587 

General  Scheme  of  Injection. — The  following  general  scheme  is 
suggested : 

1.  The  site  usually  chosen  is  the  posterior  third  of  the  buttock,  to 
avoid  the  sciatic  nerve  and  vessels. 

2.  The  injection  should  be  made  deeply  into  the  muscle,  using  each 
buttock  alternately. 

3.  The  syringe  and  piston  should  preferabh'  be  of  glass,  easily 
sterilized,  and  the  needle  of  platinum  iridium,  about  1|  inches  in 
length,  ^nd  sterilized. 

4.  The  skin  should  be  scrubbed  with  ether  soap,  washed  with  freshly 
boiled  water,  and  swabbed  over  with  an  antiseptic  solution. 

5.  After  insertion  of  the  needle,  the  piston  should  be  slightly  with- 
drawn, and  if  any  blood  appears  the  needle  should  be  reinserted  in 
order  to  avoid  injection  into  a  bloodvessel. 

Solutions. — The  injection  of  the  solution  free  from  air  bubbles  can 
then  take  place. 

Various  solutions  are  in  use.    Only  a  few  can  be  mentioned  here. 

I^ — Hydrargyri  sozoidolate        ....  gm.       0.2  gr.  ii.j 

Sodii  iodidi gm.       0.-3  gr.  v 

Aq.  dest gm.  10.0.  Jiiss 

Dose — 10  to  20  minims  constitute  the  dosage. 

I^ — Hydrargj-ri  lactatis gm.       0.2  gr.  iij 

Aq.  dest c.c.      18.0  3iv 

Dose — 10  to  25  minims. 

R — Hydrargyri  succinamidi       ....  gm.       0.2  gr.  iij 

Aq.  dest c.c.      10.0  giiss 

Dose — 10  to  25  minims. 

I^ — Hydrargyri  chloridi  corrosi\'um      .      .  gm.       0.5  gr.  viij 

Sodii  chloridi       .      ". gm.       .3.0  gr.  xlv 

Aq.  dest gm.  100.0  Biij 

Dose — 1  to  2  c.c.  dailj^  or  alternate  days. 

The  use  of  corrosive  sublimate — following  Lewin — is  usually  very 
painful. 

R — Hydrarg^T-i  cyanidi gm.  1.0  gr.  xv 

Cocaine  hydrochloridi gm.  0.3  gr.  v 

Aq.  dest ad     gm.  100.0  giij 

Dose — 1  to  2  c.c. 

A  useful  variant  of  this  combines  the  cyanide  with  arsenic  and 
strychnin,  as  follows: 

I^ — Hydrargyri  cyanidi, 

Strych.  arsenatis aa     gm.      0.6     aa     gr.  ix 

Cocain  muriat.  gm.       0.3  gr.  v 

Aq.  dest gm.    60.0  ^ij 

Dose — 5  to  10  minims  every  other  day  for  20  to  25  doses. 


588  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

Cocain  may  be  added  to  any  of  the  soluble  salts.  Its  addition 
lessens  the  pain. 

Fournier  has  always  advocated  the  use  of  the  biniodide  dissolved 
either  in  sterilized  oil  or  in  water.  It  is,  he  claims,  painless,  sure,  and 
free  from  dangers.  In  2457  injections  only  9  produced  pain.  Such 
results,  however,  obtain  only  when  the  physician  is  very  careful. 
Careless  use  with  the  biniodides  will  produce  all  of  the  accidents,  pain, 
abscess,  etc. 

I^ — Hydrargyri  biniodide gm.       0.4  gr.  vj 

Olive  oil  (sterilized) gm.     10.0  Siiss 

Dose — One  Pravaz  syringeful  every  other  day. 

I^ — Hydrargyri  biniodide gm.       0.2  gr.  iij 

Sodii  iodide gm.       0.2  gr.  iij 

Aq.  dest gm.     10.0  Siiss 

Dose — 1  to  2  c.c.  daily  or  on  alternate  days,  with  gradual  elevation  of  the  dose  if 
stomatitis  or  gastro-intestinal  signs  are  not  in  evidence.  Twenty  to  twenty-five  injec- 
tions constitute  a  course  of  treatment. 

I^ — Hydrargyri  benzoatis gm.       1.0  gr.  xv 

Sodii  chloridi gm.       2.5  gr.  xlv 

Aq.  dest gm.  120.0  giv 

Dose — 1  to  2  c.c.  daily  or  on  alternate  days. 

Combined  arsenical  and  mercurial  injections  were  very  much  in 
vogue  before  the  introduction  of  the  salvarsan  preparations.  One 
of  the  most  popular  of  these  has  been  the  arsenical  salicylate  or  enesol. 
This  remedy  has  been  used  widely  in  nervous  syphilis  and  often  with 
surprisingly  good  results.     Schaffer  speaks  very  highly  of  it. 

The  combined  use  of  the  cacodylates  and  of  mercury  has  been 
observed  to  give  good  results.  The  early  reports  of  optic  nerve  disease 
apparently  following  the  use  of  the  cacodylates  served  to  force  these 
salts  into  the  background.  Inasmuch  as  such  optic  nerve  changes 
apparently  occurred  in  other  than  syphilitic  patients,  it  would  not 
appear  that  they  are  to  be  interpreted  as  instances  of  those  neuroreci- 
dives  which  have  been  so  actively  discussed  since  salvarsan  has  been 
introduced. 

Salvarsan  and  Neosalvarsan. — Any  attempt  at  an  exhaustive  sum- 
mary of  the  various  reports  upon  this  remedy  in  the  treatment  of 
nervous  syphilis  would  require  a  special  volume.  A  simple  enumeration 
of  the  bibliography  alone — best  obtained  in  brief  in  Lewandowsky's 
Handhuch  der  Neurologic,  articles  by  Forster  and  Schaffer  and  others — 
in  Nonne's  discussion,  referred  to  later,  would  require  dozens  of  pages. 
Only  the  present  (1915)  drift  of  opinion  will  here  be  expressed. 

In  the  exudative,  hyperplastic,  gummatous,  and  arterial  forms 
salvarsan  is  by  far  the  most  efficient  remedy  that  we  possess  at  the 
present  time.  One  form  needs  to  be  excepted,  that  of  the  large  gum- 
mata,  for  which  surgery  alone  is  adequate.  It  would  also  appear 
that  much  larger  doses  of  salvarsan  are  required  for  nervous  syphilis 


TREATMENT  589 

than  were  used  in  the  earlier  stages  of  its  administration.  Since  the 
use  of  adequate  dosage  the  so-called  neurorecidives  have  almost  entirely 
disappeared.  It  is  the  present  trend  of  opinion  that  mercury  and 
salvarsan  combined  gives  the  best  results.  Whether  or  not  arsenic 
and  mercury,  both  active  spirochetal  drugs,  supplement  each  other  in 
this  combined  use  is  not  certain,  but  the  results  obtained  have  in  many 
instances  been  very  satisfactory. 

At  the  same  time  it  needs  to  be  observed  that  a  number  of  patients 
have  relapsed,  and  the  final  results  of  therapy  in  nervous  syphilis  have 
been  far  from  being  as  hopeful  as  had  appeared.  Because  this  has 
been  so  is  no  reason  why  it  should  remain  so.  The  most  obvious  reason 
that  stands  out  in  many  of  the  recent  discussions  that  have  taken 
place  relative  to  this  point  is  that  the  patients  have  been  insufficiently 
treated.  Finally,  salvarsan  has  not  been  long  enough  in  use  to  warrant 
anything  but  as  yet  tentative  conclusions. 

With  this  short  summary  of  conclusion,  a  few  words  may  be  said 
as  to  its  application  and  dosage.  Whether  neosalvarsan  is  to  replace 
salvarsan  or  not  cannot  yet  be  determined.  The  intravenous  applica- 
tion of  salvarsan  is  the  best  method  of  giving  it.  It  should  not  be 
given  unless  the  patient  is  under  some  sort  of  supervision — in  a  hospital 
or  remaining  in  bed — and  minute  attention  to  the  technique  is  abso- 
lutely necessary  to  avoid  certain  dangers.  It  is  highly  important 
that  fresh,  distilled  water  be  employed  if  salvarsan  is  to  be  used  intra- 
venously. 

To  kill  spirochetes  in  the  nervous  system,  however,  is  one  thing, 
and  to  overcome  the  results  of  tissue  changes  is  quite  a  different  one, 
and  this  above  all  is  the  stumbling-block  in  the  treatment  of  nervous 
tissue  syphilis.  Nevertheless,  if  nerve  tissues  have  not  been  extensively 
destroyed,  one  can  hope  for  excellent  results  by  a  proper  combination 
of  salvarsan  therapy  with  mercury. 

Salvarsan  must  be  used  in  much  larger  quantities,  however,  than  was 
at  first  thought.  At  the  end  of  this  section  the  outlines  of  an  energetic 
combined  therapy  is  given,  and  reference  may  be  made  to  those  pages 
for  the  general  indications  of  such  a  course  of  treatment.  Modifica- 
tions to  a  less  active  mercurial  salt  than  calomel  may  have  to  be  made. 
Every  patient  needs  individual  treatment. 

Notwithstanding  the  very  evident  fact  that  salvarsan  and  neosal- 
varsan are  active  spirocheticidal  drugs,  it  is  still  an  important  problem 
how  to  reach  them  in  the  nervous  system.  Careful  chemical  inves- 
tigation of  the  cerebrospinal  fluid  has  heretofore  failed  to  obtain  any 
trace  of  arsenic  when  salvarsan  has  been  given  in  the  usual  manner. 
One  may  infer  that  the  arsenic  has  become  fixed  in  some  chemical 
combination  which  fails  to  react  to  the  usual  chemical  tests.  It  is 
not  yet  comprehensible  why  nervous  syphilis  is  so  resistant  to  treat- 
ment and  why  the  hopes  aroused  by  the  striking  results  of  salvarsan 
therapy  in  general  syphilis  seem  not  to  have  been  borne  out  in  nervous 
syphilis. 


590  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

Swift  and  Ellis  have  attempted  to  place  a  spirocheticidal  solution 
directly  into  the  cerebrospinal  fluid.  Salvarsan  and  neosalvarsan 
were  employed  by  direct  injection  into  the  spinal  canal  through  the 
Quincke  lumbar  puncture.  This  method  they  found  was  to  be  con- 
demned. It  failed  to  give  any  beneficial  results,  and,  moreover, 
caused  marked  pains.  It  is  probably  a  dangerous  procedure,  as  animal 
experimentation  has  shown. 

An  attempt  was  then  made  to  introduce  into  the  patient's  cerebro- 
spinal fluid  some  of  his  own  blood  serum  which  had  previously  been 
mixed  with  the  salvarsan  by  intravenous  infusion.  This  procedure  is 
carried  out  in  the  usual  manner.  After  a  certain  length  of  time — one 
hour  was  found  to  give  the  most  active  serum — blood  was  withdrawn, 
separated  from  its  corpuscles,  after  twenty-four  hours,  diluted  with 
40  per  cent,  of  normal  saline,  and  then  heated  to  56°  C.  for  thirty 
minutes.  A  lumbar  puncture  is  then  made,  and  from  5  to  15  c.c.  of 
cerebrospinal  fluid  is  withdrawn,  i.  e.,  until  the  pressure  falls  to  30 
mm.  of  mercury.  Thirty  cubic  centimeters  of  the  warmed  serum  is 
then  injected  into  the  subarachnoid  spaces.  The  patient  must  lie 
quiet,  the  foot  of  the  bed  usually  being  raised.  After  ten  days  to  two 
weeks  the  injections,  which  are  usually  well  borne  are  to  be  repeated. 

They  thus  obtained  very  striking  results  in  the  action  upon  the  sero- 
biological  factors  known  to  accompany  cerebral  syphilis.  Then  a  more 
crucial  experiment  was  planned.  This  consists  in  the  introduction 
into  the  subarachnoid  spaces  of  serum  taken  from  another  individual, 
usually  a  secondary  syphilitic  under  treatment.  The  technique  being 
that  just  outlined.  In  the  treatment  of  tabes  by  a  heterologous 
serum  most  excellent  results  have  been  obtained  in  some  cases.  In 
others  they  have  been  nil  or  positively  dangerous.  Why  the 
striking  character  of  the  results,  for  it  is  at  once  evident  that  the 
amount  of  spirocheticidal  substance  in  a  few  cubic  centimeters  of 
serum  taken  from  the  body  of  another  patient  who  had  received 
the  usual  intravenous  salvarsan  therapy  must  be  very  small 
indeed,  i.  e.,  reckoned  as  arsenic?  If  other  factors  than  the  sal- 
varsan itself  enter  into  the  situation  these  are  as  yet  unknown.  One 
significant  fact,  however,  would  tend  to  indicate  that  other  forces  are 
operative.  Extensive  experiments  carried  on  by  Swift  and  Ellis  with 
the  heated  and  unheated  serums  show  that  the  heated  sera  are  three 
times  as  spirocheticidal  to  Spirocheta  duttonii  in  mice. 

This  would  raise  the  question  whether  or  no  the  heating  caused 
alterations  in  the  salvarsanized  serum  which  were  advantageous  in 
the  therapy. 

The  most  striking  suggestive  result  is  an  almost  immediate  diminu- 
tion in  the  number  of  pathological  cells  in  the  cerebrospinal  fluid.  The 
globulin  reaction  diminishes,  positive  Wassermanns  of  the  cerebrospinal 
fluid  with  small  quantities  of  fluid  require  larger  quantities  to  show 
positive  or  become  negative,  and  the  amelioration  of  the  symptoms 
has  in  a  few  cases  been  rapid. 


TREATMENT  591 

Mercury  hy  the  Mouth. — Mercury  by  the  mouth  will  always  remain 
one  of  the  simplest,  and  yet,  at  the  same  time,  least  efficient  methods 
of  treating  syphilis  of  the  nervous  system.  Here  again  one  has  a  rich 
choice  of  remedies.  Those  most  in  use  are:  protoiodide  grain  |  to 
\,  sublimate  grain  -^-q,  calomel  2  to  5  grains.   Various  vehicles  are  used. 

The  disadvantage  of  treatment  by  means  of  the  intestinal  canal 
are  many^chief  of  which  is  the  slow  and  weak  action  of  the  remedies 
employed.     Moreover,  the  gastro-intestinal  tract  suffers. 

The  chief  advantage  is  that  mercury  may  be  combined  with  the 
iodides.  Furthermore,  conveniences  of  medication  must  often  con- 
strain one  to  use  this  mode  of  giving  antisyphilitic  remedies,  but  only 
as  a  necessary  choice. 

In  nervous  syphilis  it  would  appear  that  mercury  medication  by 
mouth  is  not  radical  enough.  One  may  use  it  after  an  energetic 
treatment,  by  the  methods  outlined,  has  been  employed,  but  oral 
administration  is  rarely  a  method  of  certain'  value,  and  hence  is  not 
advisable,  save  under  particular  circumstances. 

Among  the  newer  mercury  preparations  which  future  experience 
may  prove  to  be  of  value  are:  Mercury  dicarboxylate,  two  of  which 
are  on  the  market,  with  pronounced  toxic  action  on  spirochetes  in 
rabbits.  It  is  claimed  to  be  twenty  times  as  toxic  to  spirochetes  as 
corrosive  sublimate,  and  yet  shows  no  action  on  the  body.  Its  dosage 
has  not  yet  been  worked  out. 

Iodides.— '^o6i\im  and  potassium  iodide  have  been  used  in  the  treat- 
ment of  syphilis  of  the  nervous  system  for  years,  and  often  with  good 
results.  According  to  Neisser  the  iodides  are  weak  spirochetal  poisons. 
Our  belief  in  its  resorptive  powders  is  justified  on  empirical,  if  not  on 
pharmacological  grounds.  Personal  experience  does  not  confirm  the 
belief  in  the  efficiency  of  specially  large  doses,  although  that  is  the 
American  preference. 

The  use  of  tlie  iodides  in  doses  of  from  10  to  30  grains  t.  i.  d.  com- 
bined with  mercury  is  particularly  valuable  in  the  gummatous  type 
of  cerebral  syphilis.  It  is  folly,  however,  to  try  to  do  away  with  large 
gummata  by  means  of  massive  doses  of  iodides. 

The  dosage  of  the  iodides  will  depend  upon  the  individual.  There 
are  many  idiosyncrasies  to  be  borne  in  mind.  At  times  small  doses 
cause  marked  disturbances  and  cannot  be  borne.  Here  one  may 
employ  other  combinations  than  those  of  sodium  or  potassium.  Hence, 
strontium,  rubidium,  and  organic  iodin  preparations  have  come  into 
use.  lodopin,  sajodin,  iodoglidin,  iodoval,  iodocitin,  iodostarin  are 
among  the  newer  of  these  combinations. 

lodopin  may  be  injected  as  well  as  administered  by  the  mouth.  In 
the  former  case  it  is  used  in  quantities  of  10  c.c.  on  alternate  days, 
or  smaller  doses  1  to  3  c.c.  at  more  frequent  intervals.  In  giving  it  by 
hypodermic  both  the  syringe  and  the  remedy  should  be  slightly 
warmed,  the  needle  should  have  an  ample  bore,  and  the  drug  be  intro- 
duced slowly.    It  is  also  given  by  the  mouth  in  5  j  doses.    In  the  form 


592  SYPHILIS  OF   THE  NERVOUS  SYSTEM 

of  iodopin,  large  quantities  of  iodin  may  be  introduced  without  toxic 
effect.  Its  action  on  nervous  syphilis  has  not  been  extensively  studied. 
Good  results  are  reported  by  its  use  in  syphilitic  labyrinthitis. 

lodoval  and  iodocitin,  the  latter  a  lecithin  albumin  compound,  have 
been  found  to  be  borne  well  in  the  course  of  salvarsan-mercurial  treat- 
ment. The  former  is  given  in  doses  of  about  5  grains  t.  i.  d.  throughout 
an  energetic  salvarsan-mercury  treatment — the  latter  in  about  the 
same  doses. 

Plan  of  Intensive  Treatment. — Nervous  syphilis  is  treated  too  gin- 
gerly by  most  practitioners.  It  is  difficult  to  kill  the  syphilis  organ- 
ism, hence  an  energetic  course  of  treatment  is  here  outlined: 

First  day 0 .  03  calomel  (or  other  mercurial)  hypodermically. 

Third  day 0 .  05  calomel  (or  other  mercurial)  hypodermically. 

Fifth  day 0.4    salvarsan  intravenously. 

Seventh  day     .      .      .      .  0.5    salvarsan  intravenously. 

Ninth  day ,  0 .  05  calomel  hypodermically. 

Eleventh  day    .  .      .  0 .  05  calomel  hypodermically. 

Thirteenth  day       .      .       .  0.4    salvarsan  intravenously. 
Fifteenth  day   .       .       .       .0.5    salvarsan  intravenously. 

Seventeenth  day    .      .      .  0 .  05  calomel  hypodermically. 

This  should  be  continued  for  a  six  weeks'  cure,  or  until  at  least 
5  gms.  of  salvarsan  are  administered.  The  whole  cure  can  be  com- 
pressed into  three  weeks  if  a  soluble  mercury  salt  is  given,  and  at 
least  5  gms.  of  salvarsan  can  be  administered  in  that  time.  The 
patient  should  be  watched  very  carefully,  especially  with  reference 
to  the  kidneys.  Furthermore,  there  are  patients  who  do  not  bear 
calomel  well.  Vagotonic  individuals  react  excessively  to  mercury, 
especially  to  minute  doses.  (Compare  article  on  Syphilis  of  the  Nervous 
System  in  Modern  Treatment  of  Nervous  and  Mental  Diseases,  White 
and  Jelliffe,  Vol.  II.) 


PART  III. 

PSYCHICAL  OR  SYMBOLIC  SYSTEMS. 


CHAPTER  XVI. 
THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES. 

Introduction. — The  field  of  the  neuroses  and  the  psychonein'oses 
is  not  only  the  broadest  field  in  psychiatry,  but  perhaps  the  broadest 
field  in  all  medicine.  Not  only  is  the  field  an  extensive  one  in  point  of 
the  actual  number  of  persons  who  suffer  from  these  afflictions,  but  it 
is  a  field  of  very  great  importance  for  the  understanding  of  mental 
phenomena  both  in  the  realm  of  disease  and  in  the  realm  of  the 
healthy.  It  is  in  the  manifestations  of  these  disorders,  which  have 
been  well  termed  borderland  states,  that  one  can  find  and  can  study 
the  early  departures  from  the  normal,  which,  in  much  more  aggra- 
vated form,  appear  in  the  psychoses. 

Then  again  problems  that  lend  themselves  much  more  satisfactorily 
to  therapeutic  attack  are  to  be  found  here.  Thus  we  are  dealing  with 
conditions  which,  although  they  may  represent  practically  any  degree 
of  departure  from  the  average,  in  the  main  represent  lesser  or  moderate 
degrees  of  departure  from  the  normal  which  are,  as  a  rule,  capable  of 
material  alleviation,  if  not  actual  cure  by  therapeutic  measures.  When 
one  considers  the  immense  number  of  people  who  are  affected  by 
neuroses  or  psychoneuroses,  the  great  amount  of  suffering  that  these 
diseases  entail,  the  impaired  efficiency  in  which  they  result,  and  then 
consider  that  they  are,  for  the  most  part,  susceptible  of  great  improve- 
ment, if  not  actual  cure  by  therapeutic  endeavor,  it  will  be  seen  that 
this  department  of  medicine  is  not  only  the  most  attractive,  but  is 
one  which  perhaps  offers  most  in  the  way  of  results. 

The  number  of  people  actually  afflicted  with  these  conditions  is 
difficult  to  estimate.  The  frank  cases  of  the  psychoneuroses  and  the 
actual  neuroses  are  very  numerous,  as  are  also  more  or  less  larvated 
conditions,  while  on  the  other  hand  every  specialist  in  medicine  is 
dealing  constantly  with  manifestations  of  these  conditions  as  they 
appear  upon  the  physical  side.  Perhaps  these  physical  manifestations 
are  best  known  to  the  gastro-enterologist,  the  gynecologist,  and  the 
genito-urinary  surgeon,  but  the  ophthalmologist,  the  laryngologist, 
the  internist,  and  in  fact  every  specialist  has  his  share. 
38 


594  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

The  wide  distribution  of  the  neuroses,  but  more  particularly  of  the 
psychoneuroses,  can  best  be  understood  if  certain  psychological  con- 
siderations are  first  briefly  gone  into. 

The  baby  in  its  mother's  uterus  has  no  desires;  it  has  to  do  nothing 
for  itself,  not  even  to  breathe;  it  rests  quietly,  far  removed  from  sources 
of  outside  stimulation  and  irritation,  every  function  being  performed 
for  it  by  the  mother.  After  the  baby  is  born  this  condition  of  affairs 
still  continues,  or  at  least  an  effort  is  made  for  it  still  to  continue.  The 
baby,  to  be  sure,  has  to  begin  to  breathe  for  itself,  to  eat  for  itself, 
to  perform  the  functions  of  digestion  and  elimination  for  itself,  but 
on  the  other  hand,  there  stand  about  the  army  of  the  household,  not 
satisfied  to  wait  upon  desire,  but  with  every  heartstring  of  emotion 
tense  to  forestall  it.  Everything  possible  in  the  way  of  the  baby's 
needs  is  anticipated.  He  is  waited  upon  hand  and  foot  by  all;  he  is, 
in  the  sense  that  every  desire  is  satisfied,  truly  omnipotent. 

As  the  days  go  by  and  development  proceeds  apace,  as  the  sense 
organs  become  more  acute,  the  muscular  adjustments  more  refined, 
the  baby's  contact  with  the  world  becomes  progressively  and  increas- 
ingly complex,  and  try  as  they  will  the  loving  attendants  cannot  fore- 
stall all  of  his  desires,  and  there  come  times  when  food  is  not  offered 
at  the  instant  it  is  needed,  when  sleepiness  overtakes  the  baby  but  he 
cannot  woo  it  if  he  is  in  a  bright  and  noisy  street  or  on  a  clattering 
car  far  from  his  soft  bed.  And  so  there  arises  insidiously  but  neces- 
sarily the  mental  state  of  desire,  things  wished  for  because  they  are 
not  had. 

Still  even  in  this  stage  of  development  the  discrepancy  between 
desire  and  attainment  is  not  great.  Attainment,  in  fact,  is  usually 
very  near  at  hand,  the  hunger  is  not  permitted  to  last  long,  the  baby 
does  not  have  to  be  kept  awake  by  noises,  except  for  a  brief  period, 
while  in  the  matter  of  other  desires,  such  for  instance,  as  the  desire 
to  empty  the  bladder,  that  is  indulged  in  forthwith  without  any 
further  consideration  of  the  matter.  As  development  progresses,  how- 
ever, desires  become  more  and  more  numerous,  because  the  baby 
touches  reality  at  more  numerous  points,  and  each  one  of  these  points 
offers  a  new  possibility  for  a  frustrated  or  delayed  desire,  while  with 
such  matters  as  emptying  the  bladder  there  soon  steps  into  the  situa- 
tion the  social  repressions  represented  by  the  prohibitions  of  the 
mother. 

Thus  growing  up  in  the  life  of  the  baby,  beginning  even  in  the  earliest 
days,  an  ever-increasing  discrepancy  between  desire  and  attainment 
takes  place,  and  as  the  years  go  on  it  will  be  seen,  without  the  necessity 
for  further  illustrations,  that  the  amoral,  egocentric  baby  must  grad- 
ually take  into  consideration  the  world  about  him.  He  is  forced  to 
lay  his  conduct  along  certain  lines  which  imply  a  putting  off  of  the 
satisfaction  of  desire  into' an  ever-receding  future.  Later  in  life,  when 
he  is  hungry  and  wishes  to  eat,  he  can  only  satisfy  this  desire  provided 
he  has  worked  and  earned  the  wherewithal  to  buy  food,  and  if  he  en- 


INTRODUCTION  595 

deavors  to  satisfy  it  otherwise  by  taking  any  food  that  may  be  at  hand 
he  offends  the  social  usages  and  becomes  a  thief.  If  he  wishes  to  empty 
his  bladder  he  has  to  wait  until  he  gets  to  an  appropriate  place;  it 
cannot  be  done  anywhere  and  at  any  time.  He  has  to  adjust  himself 
to  the  requirements  of  society  or  run  serious  risks  if  he  fails.  As  he 
becomes  progressively  more  complex,  as  his  desires  become  more 
and  more  difficult  of  fulfilment,  as  he  demands  more  and  more  of  the 
world,  the  individual  finds  that  he  has  to  put  off  fulfilment  further 
and  further  into  the  future  and  be  satisfied  to  struggle  perhaps  for 
years  to  attain  some  specific  end. 

Confiict  is  therefore  at  the  very  basis,  the  very  root  of  mental  life; 
the  adjustment  of  the  individual  to  the  world  of  reality  is  by  no  means 
the  passive  molding  by  external  forces,  but  the  individual  is  constantly 
and  actively,  in  his  mind  at  least,  reaching  out  and  trying  to  mold  the 
world  to  suit  himself. 

It  is  from  this  basal  fact  of  conflict  that  there  take  origin  two 
forms  of  thinking,  an  understanding  of  which  is  of  great  importance 
for  the  comprehension  of  the  psychoneuroses,  in  fact  for  all  behavior, 
sick  or  well.  Thinking  which  is  dominated  by  the  reality  motive,  the 
thinking  which  is  a  conscious  intentional  effort  at  efficient  relation 
with  reality,  is  the  thinking  to  which  the  word  thinking  is  usually 
applied.  But  there  is  another  kind  of  thinking,  the  thinking  by 
phantasy  formation,  which  is  of  great  importance.  In  this  form  of 
thinking  it  is  not  the  reality  motive  that  dominates,  but  the  pleasure- 
pain  motive.  The  other  horn  of  the  conflict  is  here  represented,  and  in 
moments  of  quiescence  when  the  real  world  slips  away  from  our  vision 
and  we  settle  back  within  ourselves,  our  thoughts  flow  without  refer- 
ence to  this  outside  world,  they  come  and  go  without  critique  on  our 
part.  We  are  dreaming,  perhaps  in  sleep  or  perhaps  in  waking,  and 
these  fancies  which  come  at  these  moments  of  rumination  are  all  wish- 
fulfiling  fancies  controlled  by  the  pleasure  motive  and  represent  the 
satisfaction  of  desires  which  are  either  put  off  or  rendered  incapable 
of  fulfilment  in  the  real  world.  These  thoughts  are  not  only  the 
thoughts  that  dreams  are  made  of,  but  the  thoughts  which  the  psy- 
choneuroses are  made  of,  and  are  therefore  of  immense  importance 
for  their  understanding. 

From  the  very  first  the  immediate  satisfaction  of  desire  is  frus- 
trated, to  be  technical  it  is  repressed  and  some  other  form  of  activity 
has  to  be  substituted,  for  example  in  later  life,  to  use  our  same  illus- 
tration, instead  of  maintaining  the  immediate  relationship  between 
hunger  and  food,  there  is  introduced  another  series  of  factors,  repre- 
sented by  work  and  compensation  for  work  in  the  shape  of  money, 
which  money  may  be  exchanged  for  food.  And  so,  instead  of  the  im- 
mediate relationship  that  maintains  in  infancy  a  more  remote  relation- 
ship is  maintained,  and  the  activities  instead  of  going  straight  to  their 
goal  take  a  more  or  less  circuitous  and  involved  path.  The  original 
relationship  therefore  tends  to  be  lost  sight  of,  and  the  more  involved 


596  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

and  complicated  one  takes  its  place.  There  are,  therefore,  gradually 
throughout  the  period  of  development,  all  sorts  of  desires  being 
repressed  which,  thus  put  out  of  consciousness,  are  replaced  by  other 
forms  of  activity.  The  desires  which  belong  to  infancy  and  which  thus 
are  early  repressed  and  substituted  by  other  forms  of  activity,  consti- 
tute the  material  out  of  which  the  unconscious  is  formed  and  the 
material  from  which  come  the  activating  moments  for  phantasy  for- 
mation. The  discrepancy  between  desire  and  fulfilment,  then,  is 
compensated  in  later  life  by  the  wish-fulfiling  phantasies  that  have 
their  origin  in  the  repressed  material  of  infancy  and  occupy  the  realm 
of  the  unconscious. 

Between  this  realm  of  the  unconscious,  which  contains  relatively 
infantile  material  only,  and  the  realm  of  the  clearly  conscious,  there  lies 
the  realm  of  the  so-called  fore-conscious,  which  contains  the  material  of 
recent  experience,  material  which  is  quite  easily  made  conscious.  In 
other  words,  it  is  just  out  of  mind  and  it  is  not  difficult  to  bring  back 
into  the  focus  of  attention  when  the  individual  so  desires. 

The  region  of  the  unconscious  is  of  very  great  importance  for  an 
understanding  of  the  psychoneuroses,  because  it  represents  the  region 
of  the  deepest  repressions,  of  the  thoughts  that  are  least  like  the  present 
conscious  thoughts,  and  which,  therefore,  when  they  break  through 
into  consciousness,  produce  symptoms  that  are  so  grotesque  and 
strange  appearing  upon  the  surface  and  non-understandable,  not  only 
to  the  onlooker,  but  to  the  patient  himself.  It  is  therefore  desirable 
to  know  somewhat  of  the  nature  of  the  unconscious  and  of  its  content. 
To  do  this  certain  features  in  the  development  of  the  child,  particularly 
those,  of  course,  that  are  at  a  later  date  repressed  and  substituted  by 
other  activities,  will  have  to  be  described. 

During  the  early  infancy  of  the  child  the  child's  love  is  very  natu- 
rally given  out  to  the  only  people  to  all  intents  and  purposes  who 
constitute  his  milieu,  namely  the  members  of  the  immediate  family, 
the  father,  the  mother,  the  brother,  sister,  and  perhaps  nurse.  This 
love,  contrary  to  the  usual  way  of  thinking  of  it,  is  very  definite  in  its 
direction,  and  from  a  very  early  date  presents  certain  sexual  character- 
istics. Of  these  sexual  characteristics  jealousy  of  a  younger  brother 
or  sister  who  comes  into  the  family  and  deflects  a  certain  amount  of 
affection  which  the  child  would  otherwise  enjoy  is  within  the  observa- 
tion of  most  people,  while  the  fact  that  the  love  of  the  child  is  given 
out  to  the  members  of  the  family,  characteristically  the  parent  of  the 
opposite  sex,  is  not  a  matter  of  such  common  observation,  but  a 
matter  of  great  importance  psychologically.  As  the  child  develops 
these  loves  are  repressed  and  covered  into  that  all  inclusive  amnesia 
for  the  infantile  period,  and  when  adulthood  comes  along  and  the  child 
has  grown  to  manhood  or  womanhood  and  finds  its  mate,  the  love 
which  had  before  been  spent  upon  the  members  of  the  family  now  finds 
its  true  object. 

It  is  this  infantile  love  for  the  members  of  the  family  that  is  the  root 


INTRODUCTION  597 

for  so  many  of  the  incest  phantasies  of  the  psychoneuroses  and  the 
psychoses.  This  love  for  the  parent  of  the  opposite  sex,  for  example, 
if  it  breaks  through  into  the  clear  realm  of  consciousness  becomes  a 
horrid  thing  incompatible  with  the  individual's  peace  of  mind.  Such 
things  are  quite  common.  For  example,  a  patient  marries  a  man 
who  unfortunately  presented  a  number  of  very  close  resemblances 
to  her  father.  These  resemblances  served  to  stir  into  activity  the 
unconscious  love  for  the  father,  and  she  therefore,  in  her  feelings  toward 
her  husband,  is  outraged  beyond  all  endurance,  for  it  is  as  if  she  were 
married  to  her  father.  Life  with  her  husband  is  quite  unendurable. 
She  is  constantly  flying  into  passions,  assaulting  him,  upbraiding 
him,  etc. 

If  this  psychology  is  the  usual  psychology,  why  is  it  that  all  people 
are  not  in  danger  from  such  sources?  Perhaps  they  are  to  a  limited 
degree,  but  it  is  necessary  to  bear  certain  things  in  mind  to  under- 
stand how  the  unconscious  becomes  mixed  up,  as  it  were,  in  the  daily 
life  of  the  individual,  as  in  the  case  just  cited.  An  individual  with  such 
an  unconscious  father  complex  will  get  along  in  life  perhaps  q'uite 
well  until  they  meet  some  difficulty.  The  difficulty  drives  them  back 
within  themselves,  it  prevents  the  outward  flow  of  interest  into  reality, 
makes  them  egocentric,  introspective,  they  are  unable  to  make  an 
efficient  reaction,  and  they  therefore  are  driven  back  to  phantasy 
formation  where  things  come  true  and  the  difficulties  are  all  removed. 
The  reason  why  this  driving  back  of  the  psychophysical  energy  within 
the  individual  under  conditions  of  stress,  the  reason  w^hy  this  should 
stir  up  a  particular  complex,  is  because  in  the  life  of  the  individual 
there  has  been  an  undue  fixation  at  that  point  in  the  course  of  develop- 
ment. This  patient  just  cited  had  never  been  able  to  emancipate 
herself  as  she  should  have  from  the  necessity  for  the  loving  care  and 
tenderness  and  protection  of  the  father  and  to  go  out  into  the  world 
and,  so  to  speak,  stand  upon  her  own  feet,  and  when  difficulties  arose 
in  her  life  and  she  was  thrown  back  upon  herself,  she  went  back  to 
that  point  at  which  there  had  been  an  infantile  fixation. 

From  the  few  words  of  description  of  this  patient's  condition,  who 
had  symbolically  married  her  father,  it  will  be  seen  how  important  it 
becomes  to  know  the  content  of  the  phantasies,  and  this  is  best  deter- 
mined by  a  study  of  the  dreams,  and  without  going  into  the  principles 
of  dream  analysis,  which  are  out  of  place  here,  it  is  well  to  remember 
that  the  neurosis  or  the  psychoneurosis,  like  the  dream,  is  not  only  a 
compromise  between  desire  and  fulfilment,  but  it  is  a  wish-fulfiling 
mechanism  that  brings  to  pass  the  fulfilment  both  of  the  w^ish  in  the 
foreconscious,  the  wish  with  reference  to  the  difficulty  that  caused  the 
introversion  in  the  first  place,  and  also  the  wish  in  the  unconscious, 
the  wish  at  the  fixation-point,  which  serves  as  a  pull-back  once  the 
introversion  has  started.     (See  Psychoanalysis  in  Chapter  II.) 

Bearing  these  facts  in  mind  it  will  be  easy  to  understand  that  the 
child's  first  sexual  feelings  have  reference  to  its  own  body,  it  is  auto- 


598  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

erotic;  that  next  its  sexual  feelings  are  transferred  upon  those  imme- 
diately about  him,  upon  someone  most  like  himself,  therefore  of  the 
same  sex  (homosexual  stage).  It  seeks,  in  other  words,  outside  of 
itself,  but  still  an  object  as  much  like  itself  as  possible.  And  finally, 
the  period  of  object  love,  when  fulfilment  is  had  in  an  entirely  different 
individual  and  of  a  different  sex  (heterosexual  stage). 

In  addition  to  the  above  facts  the  child  not  only  passes  through  these 
various  stages  of  psychosexual  development  mentioned,  but  in  its 
earliest  infantile  state  it  is  susceptible,  theoretically  at  least,  of  deflec- 
tion in  any  direction.  So,  for  example,  at  the  period  when  the  love  is 
given  out  to  those  in  the  immediate  surroundings  it  not  infrequently 
is  given  out  to  a  member  of  the  same  sex;  differences  in  sex  are  not 
appreciated  in  these  early  days  and  come  only  with  later  development. 
Other  differences  are  equally  indefinite.  The  erogenous  zones  of  which 
the  genital  organs  are  only  one,  and  the  anus  and  the  lips  constitute 
the  most  important  additional  ones,  are  still  more  or  less  indefinite, 
and  sexual  erethism  may  be  predominantly  focalized  in  any  one  of 
them.  And  so  the  roots  of  the  various  so-called  perversions  are  found 
in  these  early  fixations.  The  determining  factor  in  the  early  fixations, 
the  mechanisms  that  have  brought  them  about,  in  short,  their  uncov- 
ering, can  only  be  accomplished  by  fathoming  the  unconscious.  This 
is  the  work  of  psychoanalysis  and  the  most  prominent  means  at  its 
disposal  is  by  the  analysis  of  dreams.  (See  Chapter  II  on  Mental 
Examination.) 

THE  PSYCHONEUROSES. 

Hysteria. — Historical. — To  write  the  history  of  hysteria  would  mean 
practically  to  write  the  history  of  medicine,  for  hysteria  stands  through- 
out the  ages  as  the  type  of  functional  disturbance  of  the  nervous 
system  which,  protean  in  its  manifestations,  is  found  associated  with 
all  great  therapeutic  movements  in  medicine.  Whether  it  be  the  thera- 
peutics of  religious  conversion,  of  Perkin's  tractors,  or  hypnotism,  or 
more  recently  of  persuasion,  a  considerable  proportion  of  the  patients 
who  recover  and  thereby  become  largely  responsible  for  the  vogue  of 
the  particular  therapeutic  measure  involved,  belong  to  the  great 
clinical  group  of  hysteria. 

Hysterical  manifestations  have  been  prominent  in  mental  epidemics 
that  have  swept  over  whole  continents,  while  the  more  specific  and 
the  more  grotesque  symptoms  have  always  been  observed  and  described. 

The  modern  period  in  the  history  of  hysteria  might  be  said  to  have 
begun  with  Charcot.  This  period  is  still  so  recent  as  to  be  within  the 
memory  of  many,  and  the  influence  which  the  Charcot  school  exerted 
is  still  all  too  dominant  in  certain  quarters.  The  picture  of  hysteria 
as  Charcot  drew  it,  particularly  of  the  grande  hysterie  with  its  regular 
march  of  histrionic  attitudinizing,  as  set  forth  in  the  world-renowned 
pictures  of  Richer,  is  familiar. 


MECHANISM  OF  HYSTERIA  599 

For  many  years  following  Charcot  the  most  brilliant  work  in  eluci- 
dating the  hysteria  problem  was  done  in  France,  and  many  illustrious 
names  are  crowded  into  a  few  years.  All  sorts  of  explanations  were 
formulated,  theories  that  were  physiological,  that  were  psychological, 
and  that  were  biological,  with  numerous  variants  of  each.  The  most 
illuminating  worker  in  this  field  for  many  years,  the  one  whose  theories 
produced  the  greatest  influence  in  the  study  of  this  disease  was  Dr. 
Pierre  Janet  of  Paris.  His  was  a  theory  of  dissociation,  and  he  believed 
hysteria  to  be  purely  a  mental  malady.  It  was  due  to  a  poor  synthesis 
of  the  personality  which  enabled  certain  groups  of  ideas  to  drop  away 
from  effective  association  with  the  main  portion  of  the  personality 
and  occupy  a  region  which  Janet  termed  the  subconscious,  and  there 
existing  more  or  less  independently,  produce  their  results  irrespective 
of  corrections  from  the  rest  of  the  personality.  The  hysterical  manifes- 
tations, then,  were  the  manifestations  of  these  split-off  parts  of  the 
personality.  Janet's  views  were  a  great  advance  upon  the  current 
concepts  of  hysteria,  but  although  they  rendered  possible  a  deeper 
insight  into  the  nature  of  the  disease  and  the  disease  processes,  they 
were  still  largely  descriptive,  though,  of  course,  the  description  was 
much  refined  from  that  of  Charcot. 

Various  kinds  of  dissociation  theories  have  been  built  up  by  investi- 
gators since  Janet,  and  the  dissociation  theory  was  variously  elaborated, 
particularly  in  this  country  by  Sidis,  White,  Prince  and  others.  It 
remained,  however,  for  a  Viennese  physician,  Sigmund  Freud,  to  get 
beyond  the  point  of  description  into  a  true  interpretative  attitude 
toward  the  disease. 

Freud  showed  that  the  reason  for  the  dissociation  was  that  the  dis- 
sociated ideas  were  out  of  harmony  with  the  rest  of  the  personality,  that 
they  represented  ideas  that  were  in  conflict  with  the  ideas  forming 
the  consciousness  of  the  individual,  and  that  they  were  therefore 
repressed.  Repression  became  with  Freud,  then,  the  fundamental 
factor  at  the  basis  of  hysterical  manifestations,  an  active,  not  a  passive, 
mental  factor  which  tended  to  put  out  of  mind  certain  inacceptable 
groups  of  ideas,  and  was  therefore  the  cause  of  the  dissociation. 

The  Mechanism  of  Hysteria. — Starting  with  dissociation  as  the  most 
fundamental  descriptive  term  applicable  to  the  hysterical  state — the 
doubling  of  the  personality,  in  the  sense  of  Janet — it  has  been  seen  that 
there  is  at  the  basis  of  this  process  of  dissociation  an  active  process 
called  repression,  which  has  as  its  function  the  splitting-off  of  inac- 
ceptable idea  constellations — complexes — from  the  main  body  of  the 
personality,  and  thus,  so  to  speak,  putting  them  out  of  mind.  It 
has  also  been  intimated  that  these  split-off  complexes,  because  of 
being  split  off,  do  not  therefore  cease  to  act.  As  a  matter  of  fact  they 
go  on  functioning,  but  the  functioning  is  independent,  more  or  less, 
of  the  balance  of  the  personality. 

This  process  of  repression  and  dissociation,  following  upon  conflict, 
is  a  very  general  one  and  is  found  in  divers  mental  states,  and  is  in 


600  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

fact  a  normal  process.  It  is  not  these  processes  or  mechanisms  which 
are  characteristic  of  any  particular  mental  disorder,  but  it  is  the 
way  in  which  the  split-off  complexes  manifest  themselves  that  produces 
the  different  types  of  mental  disorders. 

From  what  has  been  said  it  will  be  seen  that  if  the  individual,  or 
more  specially,  the  psyche,  be  considered  as  being  a  complex  of  adaptive 
mechanisms  which  is  always  making  an  effort  to  come  into  closer 
adaptation  with  the  environment,  then  the  meaning  of  a  conflict  is 
that  there  enters  into  this  mechanism  certain  factors  to  which  it  can- 
not make  efficient  adaptation.  This  results  in  repression  and  splitting, 
but  the  whole  tendency  of  the  machine  is  to  readjust  effectively  by 
bringing  about  in  some  way  a  new  state  of  affairs.  In  the  conflict 
there  are  two  groups  of  tendencies  in  the  psyche  which  are  diametri- 
cally opposed  one  to  the  other.  No  solution  of  the  conflict  can  possibly 
be  brought  about  by  a  fulfilment  of  one  of  these  groups,  because  mani- 
festly the  conflict  would  still  remain.  Therefore  any  readjustment 
that  takes  place  must  in  some  way  bring  to  pass  the  tendencies  of  both 
groups.  Inasmuch  as  these  groups  are  opposed  to  each  other,  such  a 
result  cannot  actually  be  brought  to  pass  in  the  world  of  reality.  There- 
fore an  artificial  world  which  is  not  governed  by  the  strict  laws  of 
cause  and  effect  has  to  be  brought  into  existence  wherein  these  opposing 
forces  can  both,  as  it  were,  allegorically  find  their  ends  attained.  This 
is  well  shown  in  the  following  dream:  The  patient  said  "she  saw  her- 
self dead,  lying  in  a  coffin,  with  a  red  rose  in  her  hand."  The  red 
rose  symbolized  her  sweetheart  because  of  the  frequent  presents  of 
red  roses  which  he  had  made  to  her.  Being  dead  in  a  coffin  probably 
has  several  meanings,  but  among  others  has  the  meaning  of  a  regres- 
sion. The  coffin  is  the  matrix,  it  symbolizes  a  going  back  to  the  pro- 
tection of  the  mother,  and  so  the  dream  symbolizes  the  two  opposing 
desires,  one  infantile,  the  other  adult  and  recent. 

In  the  different  mental  disorders  this  end  is  brought  about  in  differ- 
ent ways.  The  hysterical  mechanism  is  different  from  the  other 
mechanisms  inasmuch  as  while  it  is  a  general  rule  that  the  painful 
affect  of  the  split-off  complexes  is  drafted  off  by  various  channels  and 
thus  finds  expression,  and  while  it  is  a  general  rule  that  this  expression 
is  not  consciously  associated  with  the  idea  content  of  the  complexes 
themselves  so  that  the  patient  is  saved  from  a  realization  of  their 
true  nature,  is  thus  conserved  from  an  appreciation  of  the  pain  that 
would  result  if  they  were  understood  at  their  true  value,  in  hysteria 
the  painful  affect  is  drafted  off  into  bodily  innervation,  thus  producing 
the  somatic  phenomena  of  hysteria.  This  is  the  process  of  conversion 
and  is  characteristic  of  hysteria.  The  so-to-speak  strangulated, 
unreacted-to  emotion  of  the  split-off  complexes  manifests  itself  as  the 
physical  symptoms  of  the  psychoneurosis  and  in  this  way  the  strong 
affect  of  the  split-off  complex  is  weakened.  The  complex  is  robbed 
of  its  affect,  which  is  the  real  object  of  conversion  and  hence  its  value 
to  the  individual. 


HYSTERIA—SYMPTOMS  601 

Symptoms.— The  symptomatology  of  hysteria  is  naturally  a  very 
complex  one,  but  from  what  has  been  said  it  will  be  seen  that  it  tends 
to  group  itself  more  especially  about  disturbances  of  motion  and  of 
sensation.  In  addition  to  this  it  also  tends  to  manifest  itself  in  certain 
crises. 

All  forms  of  paralyses  and  anesthesias  may  manifest  themselves. 
Paralyses  of  the  limbs,  either  singly  or  hemiplegia  with  or  without 
contracture,  are  common,  while  anesthesias  may  be  distributed  in 
almost  any  way,  involving  the  superficies  or  the  special  senses.  As  a 
rule,  of  course,  the  distribution  of  these  various  phenomena  do  not 
follow  the  anatomical  areas  of  nerve  supply.  They  show  some  sym- 
bolic grouping. 

The  disturbance  of  sensibility  are  of  many  forms.  Very  characteristic 
are  the  glove  and  stocking  anesthesias,  involving  the  extremities  of  the 
limbs,  hands,  a  lower  part  of  forearm  and  feet,  and  lower  portion  of  legs. 
Patches  of  anesthesia  may  be  found  upon  any  portion  of  the  cutaneous 
surface  and  they  may  be  widely  distributed  and  often  not  constant 
in  location  but  varying  with  different  examinations.  Hemianesthesia, 
especially  of  the  left  side  of  the  body,  crossed  and  alternating  forms 
are  found.  Light  touch  is  more  often  involved,  frequently  deep 
pain  also,  while  insensitiveness  to  heat  and  cold  also  occurs. 

A  characteristic  form  of  anesthesia  which  is  very  frequently  found 
is  concentric  limitation  of  the  field  of  vision. 

These  anesthesias  do  not  follow  anatomical  areas  and  experiments 
will  readily  determine  that  they  are  psychological.  If  for  example 
an  anesthetic  area  is  stimulated,  the  patient  will  say  he  feels  nothing, 
but  if  asked  to  guess  the  nature  of  the  stimulus,  will  show  a  surprising 
number  of  correct  replies. 

Hypo-esthesias,  hyperesthesias,  various  forms  of  neuralgia,  especially 
visceral,  and  headache  are  also  frequently  hysterical. 

TJie  disturbances  of  motility  are  largely  various  forms  of  paralysis. 
Hemiplegia,  monoplegia  and  paraplegia,  with  or  without  contractures, 
are  the  commoner  forms.  Astasia  abasia  is  a  characteristic  hysterical 
condition. 

Spasms  of  various  parts  are  not  infrequent.  Spasms  of  the  tongue, 
of  the  face  and  of  the  extremities  when  not  demonstrably  of  organic 
origin  are  hysterical  as  a  rule.  Tremors,  myasthenic  types  of  reaction, 
and  easy  fatigability  are  frequent.  Choreiform  movements,  tics,  and 
certain  occupation  spasms  are  often  hysterical. 

The  speech  is  involved  frequently.  Hysterical  aphonia  is  well 
known  and  usually  a  diagnosis  is  warranted  if  the  patient  can  only 
whisper  replies  to  questions  and  an  examination  discloses  healthy 
vocal  cords.  Stuttering  is  frequently  hysterical  and  an  analysis  will 
show  that  the  words  with  which  there  is  difficulty  have  especial 
significance  for  the  patient.  Other  respiratory  disturbances  of  an 
asthmatic  character  may  also  be  hysterical. 


602         THE  PSYCHONEUROSBS  AND  ACTUAL  NEUROSES 

Visceral  disturbances,  especially  of  the  gastro-intestinal  tract,  many 
of  the  false  gastropathies  with  gastric  crisis  of  vomiting  and  diarrhea, 
are  quite  frequent.  There  may  also  be  hysterical  attacks  simulating 
renal  or  hepatic  colic,  gastric  ulcer,  etc. 

Vasomotor  disturbances,  localized  edemas,  disturbed  reflexes,  fever, 
secretory  and  trophic  disorders  have  all  been  described. 

Symptoms  which  cannot  be  accounted  for  on  anatomical  and 
pathological  grounds  should  always  lead  to  an  analytic  examination 
of  the  psyche.  Even  marked  disturbances  may  have  originated 
in  the  psychic  and  continued  so  long  as  to  produce  organic  changes, 
as  for  example,  muscular  atrophy  from  prolonged  disuse  of  a  limb, 
the  paralysis  of  which  was  of  psychogenic  origin. 

Among  the  episodic  phenomena  are  found  disturbances  of  emotion, 
either  exaltation  or  depression,  which  can  be  understood  because  of 
the  displacement  of  the  affect.  There  are  various  types  of  delirium 
which  may  or  may  not  be  associated  with  convulsive  seizures,  produc- 
ing, especially,  when  long  drawn  out,  the  so-called  somnambulisms, 
during  which  all  sorts  of  ideas  may  be  manifested  and  the  patient 
be  quite  disoriented.  Dream  states  not  infrequently  occupy  the 
field  and  lead  by  development  to  all  sorts  and  degrees  of  double 
personality,  which  is  simply  a  more  elaborate  expression  of  the  split- 
off  complexes,  indicating  that  they  form  a  relatively  large  part  of  the 
personality.  In  fact,  these  split-off  systems,  provided  recovery  is  not 
possible,  tend  to  gather  to  themselves  more  and  more  of  the  person- 
ality and  thereby  to  lead  a  more  and  more  independent  and  broader 
existence. 

Amnesias  of  course  are  frequent  in  the  symptomatology.  Any 
portion  of  the  personality  which  is  active  may  be  amnesic  for  any 
portion  of  the  personality  which  is  opposed  to  it  in  the  conflict. 

In  the  analysis  of  hysterical  symptoms  one  finds  it  relatively  easier 
to  account  for  them  logically.  While  the  symptoms,  on  the  surface, 
often  resemble  dementia  precox,  one  is  not  so  often  brought  face  to 
face  with  the  crude  outcropping  of  the  unconscious.  It  is  more  fre- 
quently found  that  the  symptoms  lead  directly  back  to  actual  situa- 
tions, as  in  the  case  of^  Lucy  R.,  published  by  Breuer  and  Freud.  This 
patient  was  disturbed  by  a  subjective  sensation  of  smell,  which  was 
traced  back  to  a  smell  of  burning  pastry  in  a  perfectly  well-recollected 
scene  where  the  children  had  forgotten  the  pastry  and  it  had  become 
burnt.  Why  the  smell  of  burning  pastry  should  be  chosen  for  hyster- 
ical conversion  was  again  traced  to  the  young  woman's  love  for  the 
children  for  whom  she  was  governess  and  the  repressed  wish  that  she 
might  take  the  mother's  place  as  the  result  of  her  love  for  her  master. 
And  in  the  case  of  Freud's  of^  Elizabeth,  who,  while  engaged  in  nursing 
her  sick  father  spends  one  evening  away  from  home  at  the  solicitation 

1  Freud,  S.,  Selected  Papers  on  Hysteria  and  Other  Psychoneuroses,  Nervous  and 
Mental  Disease  Monograph  Series,  No.  4. 
^  Loc.  cit.    • 


HYSTERIA— SYMPTOMS  603 

of  her  family.  Upon  this  occasion  she  meets  a  young  man  and  on  her 
walk  home  with  him  gives  herself  up  to  the  happiness  of  the  situation. 
On  the  return,  however,  finding  her  father  much  worse,  she  bitterly 
reproaches  herself  for  forgetting  him  in  her  own  pleasure.  This 
thought,  however,  is  repressed.  In  the  course  of  her  caretaking  she 
had  each  morning  to  change  the  dressings  on  her  father's  swollen  leg. 
To  do  this  she  took  his  leg  upon  her  right  thigh.  The  suppressed 
complex  seized  upon  the  feeling  of  weight  and  pain  of  her  father's 
leg  upon  her  thigh  as  an  efiicient  avenue  of  expression  for  her  repressed 
wish  which  thus  comes  into  consciousness  under  the  disguise  of  a 
painful  area  on  the  right  thigh  corresponding  in  extent  and  location 
to  the  place  upon  which  the  father's  leg  rested. 

From  these  examples  it  will  be  seen  that  the  hysteric  is  the  victim 
of  the  spontaneous  and  aberrant  activity  of  respressed  and  split-off 
complexes  that  have  to  do  with  past  events  in  the  patient's  life  and 
that  the  expression  of  these  complexes  produces  the  symptoms  of  the 
psychoneurosis,  and  that  so  far  as  the  hysterical  manifestations  are 
concerned  the  hysteric  may  be  said  to  live  in  the  past,  for  each  access 
of  symptoms  is  but  a  reanimation  of  past  experiences. 

Like  all  psychoneurotics  the  hysteric  is  infantile.  In  other  words, 
there  is  a  certain  defect  in  psychosexual  development,  and  the  difficul- 
ties which  they  meet  in  life  tend  to  drive  them  back  upon  themselves, 
to  cause  an  introversion  of  the  libido,  that  is,  to  remove  their  interest 
from  the  actual  world  of  reality  and  to  center  it  back  again  in  them- 
selves. As  already  explained,  this  introversion  process  tends  to  reani- 
mate progressively  lower  psychosexual  levels,  and  with  a  patient  who 
is  already  infantile  the  tendency  to  reanimate,  for  example,  the  auto- 
erotic  level  is  easily  manifest.  This  is  well  shown  in  certain  symbolic 
masturbatory  acts  which  recur  during  the  hysterical  seizures  of  which 
probably  involuntary  micturition  is  one. 

The  symptomatology  of  hysteria  is  then  the  symptomatology  of  the 
activity  of  the  split-off  dissociated  idea  constellations  or  complexes  and 
their  manifestation  by  the  mechanism  of  conversion  thereby  produc- 
ing symptoms  of  physical  disorder. 

These  split-off  complexes  tend  always  to  become  dynamic  and  mani- 
fest themselves  episodically  in  the  hysterical  seizures.  The  process  of 
dissociation  or  splitting,  once  begun,  tends  to  continue  and  new  material 
tends  constantly  to  be  added  to  these  split-off  elements  by  further 
cleavage,  and  thus  this  new  portion  of  the  personality  continues  to  grow 
at  the  expense  of  the  total  personality.  Energy  accumulates  in  these 
split-off  systems,  and  when  it  becomes  sufficient  in  amount  it  breaks 
through,  so  to  speak,  and  produces  the  attacks  These  attacks  are 
made  up  characteristically  of  a  living  over  again  of  those  experiences 
which  constituted  the  etiological  moments  of  the  psychoneurosis.  In 
hysteria,  as  has  been  pointed  out,  the  breaking  through  of  the  energy 
from  the  split-off  complexes  manifests  itself  in  bodily  innervation — the 
symptoms  of  the  disease  are  physical. 


604         THE  PSYCHONEVROSES  AND  ACTUAL  NEUROSES 

Aside  from  these  episodic  manifestations,  the  crises  or  paroxysms 
of  the  disease,  there  are  the  so-called  interparoxysmal  symptoms,  which, 
harking  back  to  a  middle  age  demonology  are  still  termed  stigmata. 
These  are  most  characteristically  various  anesthesias,  anesthesias 
which  are  rarely  complained  of  by  the  patient,  often  entirely  unknown 
to  him,  being  only  brought  out  upon  examination.  It  is  because  of 
this  latter  fact  that  Babinski  has  been  led  into  the  error  of  supposing 
that  they  were  entirely  the  result  of  the  examination,  a  position  the 
erroneousness  of  which  one  can  demonstrate  to  one's  own  satisfaction. 
Even  though  it  were  absolutely  true,  the  fundamental  fact,  the  why 
of  the  symptoms,  the  reason  for  certain  patients  reacting  in  such  a 
way  to  an  examination,  remains  unexplained  by  this  renowned  French 
neurologist. 

An  analysis  of  the  stigmata  shows  also,  and  usually  without  much 
difficulty,  a  logical  connection  with  preceding  experiences,  as  for 
example,  the  smell  in  the  case  of  Lucy  R.,  or  the  anesthesia  of  the  thigh 
in  the  case  of  Elizabeth,  already  cited. 

There  is  another  group  of  symptoms  which  follow  of  necessity  as  a 
result  of  the  splitting  of  the  personality.  It  can  be  easily  seen  from 
this  dynamic  conception  of  the  nature  of  the  disease  that  a  person  who 
is  not  at  one  with  himself  has  not  at  any  one  time  the  full  quota  of  his 
energies  available,  and  therefore  it  is  found  that  the  general  efficiency 
of  this  class  of  patients,  particularly  in  the  psychic  sphere,  is  very 
greatly  reduced;  it  is  reduced  in  proportion  to  the  amount  of  the 
personality  which  is  represented  by  these  split-off  complexes.  These 
patients,  therefore,  are  not  equal  to  the  tasks  they  once  could  do. 
They  are  nervous  and  irritable,  they  tire  easily,  they  lack  capacity  for 
consecutive  application,  and  forget  readily.  These  are  general  symp- 
toms of  the  condition,  secondary  symptoms,  the  result  of  any  splitting, 
and  which  are  added  to  the  primary  symptoms  which  are  expressions 
of  the  actual  conflict. 

A  connecting  link  between  the  two  portions  of  the  personality  is 
seen  in  the  hysterical  phantasies  and  also,  of  course,  in  the  dreams 
which  themselves  belong  to  the  realm  of  phantasy  formation,  phan- 
tasies which  are  thoughts  that  come  without  being  bidden  at  moments 
of  mental  abstraction,  thoughts  that  flow  along  without  volitional 
choice,  that  replace  one  another  without  the  exercise  of  critique,  in 
other  words,  day-dreammg  or  night-dreaming  as  the  case  may  be. 
These  phantasies  represent  the  activity  of  the  submerged  complexes 
as  they  break  through  and  manifest  themselves  in  the  upper  con- 
sciousness. They  are  of  great  importance  in  discovering  the  nature 
of  the  conflict  and  are  very  common  features  of  the  hysteric,  although 
usually  the  patient  does  not  realize  it  until  his  attention  is  addressed 
to  these  vagrant  mental  manifestations,  because  they  not  only  come 
unbidden,  but  when  they  go  they  leave  no  tell-tale  traces  in  the  con- 
scious memory.  A  further  connection  between  the  unconscious  and 
conscious  are  the  conscious  phantasies.     These  are  phantasy  forma- 


COMPULSION  NEUROSIS  005 

tions  which  apparently  He  in  clear  consciousness  and  are  not  repressed. 
Phantasies  of  this  sort  are  permitted  in  clear  consciousness  only  because 
they  are  not  understood  at  their  true  value.  They  really  represent 
chiefly  repressed  material. 

To  resume,  hysteria  is  the  result  of  a  splitting  of  the  personality  in 
which  certain  split-off  complexes  are  sexually  determined,  and  leading 
an  existence  more  or  less  independent  of  the  total  personality  express 
themselves  by  the  mechanism  of  conversion  in  bodily  innervation. 
The  hysterical  symptoms,  then,  become  the  representation  through 
conversion  of  the  unconscious  phantasies  which  originate  in  the  re- 
pressed complexes,  while  the  structure  of  the  hysterical  attack  is  in 
every  way  similar  to  that  of  a  dream.  The  attack  is  the  breaking 
through  of  the  energy  of  the  repressed  systems  and  manifests  itself 
by  a  wish-fulfiling  delirium,  the  elements  of  which  may  be  over- 
determined,  displaced,  and  inverted  for  purposes  of  disguise,  as  are 
the  elements  of  a  dream. 

Compulsion  Neurosis. — Compulsion  neurosis  contains  probably  the 
majority  of  that  complex  group  to  which  Janet  gave  the  name  of  psy- 
chasthenia.  Janet's  group,  however,  contained  not  only  the  compul- 
sion neuroses,  but  a  number  of  other  things,  particularly  the  anxiety 
neuroses,  probably  many  anxiety  hysterias,  perhaps  some  neurasthen- 
ias, schizophrenias,  and  hysterias. 

The  characteristics  of  the  compulsion  neurosis  are  the  presence  in 
the  mind  of  certain  compulsive  tendencies  to  act  or  think  in  various 
ways.  The  patient  is  forced  against  his  will  and  without  apparent 
reason  to  think  certain  ideas  or  certain  thoughts  or  to  do  certain 
things.  The  compulsion  increases  until  it  is  yielded  to,  then  a  period 
of  calm  follows  which  may  be  of  variable  length,  until,  so  to  speak,  the 
energy  has  again  accumulated,  when  the  compulsion  again  manifests 
itself  and  must  again  be  relieved  by  yielding.  The  patient  has  perfect 
insight  into  the  matter,  knows  the  whole  business  is  foolish,  but  he 
cannot  help  it. 

Mechanism  of  Compulsion  Neurosis, — In  hysteria  the  repressed 
material  manifests  itself  by  conversion.  The  accumulated  affect  of 
the  split-off  complexes  is  drained  off  through  bodily  innervation.  The 
high  affect-ladened  complexes  are  thus  deprived  of  their  emotion. 
The  hysterical  attack  is  a  wish-fulfiling  delirium  which  brings  to  pass 
in  a  sort  of  allegorical  dramatization  the  fulfilment  of  both  elements 
in  the  conflict. 

In  contradistinction  to  these  characteristics  of  hysteria,  in  the 
compulsion  neurosis,  there  is  no  conversion.  The  affect  of  the  repressed 
complexes  is  drained  off,  not  through  bodily  innervation,  but  by  attach- 
ment to  otherwise  indifferent  ideas.  The  affect  is  displaced.  This 
displacement,  quite  as  in  conversion  of  hysteria,  is  a  distortion 
mechanism  and  serves  equally  with  it  to  disguise  from  the  patient  the 
real  source  of  the  affect. 


606  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

Then  again,  while  in  the  hysterical  attack  both  elements  in  the 
conflict  come  to  contemporaneous  fulfilment,  such  unification  through 
the  symptoms  is  less  evident  in  the  compulsion  neurosis,  although  the 
attempt  is  made  to  bring  it  about.  What  occurs  on  the  surface,  at 
least,  is  a  constant  alternation  between  the  ascendency  of  the  two 
factors  in  the  conflict,  which  two  factors  in  their  ultimate  analysis 
resolve  themselves,  perhaps  always,  into  love  and  hate. 

The  compulsion  neurosis  is  a  true  defense  neurosis  and  its  symptoms, 
at  least  the  compulsive  acts,  which  develop  late  in  the  course  of  the 
disorder  are  of  the  nature  of  ceremonials,  which  not  only  serve  to  dis- 
guise the  true  situation  from  the  patient,  but  so  to  speak,  atone  for 
evil. 

Freud  has  very  well  said  that  it  is  much  more  correct  to  speak  of 
obsessive  thinking  than  of  obsessive  ideas.  It  is  the  obsessive  element, 
the  compulsion,  the  so-called  Zwang  of  the  Germans  that  is  the  essential 
thing  in  this  neurosis,  and  which  may  express  itself  in  all  possible  ways. 

The  mechanism  of  the  production  of  the  symptoms  of  the  compul- 
sion neurosis  is  a  quite  complicated  one.  It  cannot  be  expressed  better 
than  by  quoting  the  language  of  Freud. ^ 

"Sexual  experiences  of  early  childhood  have  the  same  significance 
in  the  etiology  of  the  compulsion  neurosis  as  in  hysteria,  still  we  no 
longer  deal  here  with  sexual  passivity  but  with  pleasurably  accom- 
plished aggressions,  and  with  pleasurably  experienced  participation 
in  sexual  acts,  that  is,  we  deal  here  with  sexual  activity.  It  is  due  to 
this  difference  in  the  etiological  relations  that  the  masculine  sex  seems 
to  be  preferred  in  the  compulsion  neurosis. 

"  In  all  my  cases  of  compulsion  neurosis  I  have  found  besides  a  sub- 
soil of  hysterical  symptoms  which  could  be  traced  to  a  pleasurable 
action  of  sexual  passivity  from  a  precedent  scene.  I  presume  that  this 
coincidence  is  a  lawful  one  and  that  premature  sexual  aggression  always 
presupposes  an  experience  of  seduction.  But  I  am  unable  to  present 
as  yet  a  complete  description  of  the  etiology  of  the  compulsion  neurosis. 
I  only  believe  that  the  final  determination  as  to  whether  a  hysteria 
or  compulsion  neurosis  should  originate  on  the  basis  of  infantile 
traumas  depends  on  the  temporal  relation  of  the  development  of  the 
libido. 

"The  essence  of  the  compulsion  neurosis  may  be  expressed  in  the 
following  simple  formula:  Obsessions  are  always  transformed  re- 
proaches returning  from  the  repression  which  always  refer  to  a  pleasur- 
ably accomplished  sexual  action  of  childhood.  In  order  to  elucidate 
this  sentence  it  will  be  necessary  to  describe  the  typical  course  of 
compulsion  neurosis. 

"In  a  first  period — period  of  childish  immorality — the  events  con- 
taining the  seeds  of  the  later  neurosis  take  place.  In  the  earliest  child- 
hood there  appear  at  first  the  experiences  of  sexual  seduction  which 

1  Hitschmann,  Freud's  Theories  of  the  Neuroses,  Nervous  and  Mental  Disease  Mono- 
graph Series,  New  York. 


COMPULSION  NEUROSIS  607 

later  makes  the  repression  possible,  and  this  is  followed  by  the  actions 
of  sexual  aggressions  against  the  other  sex  which  later  manifest  them- 
selves as  actions  of  reproach. 

"  This  period  is  brought  to  an  end  by  the  appearance  of  the — often 
self-ripened — sexual  'maturity.'  A  reproach  then  attaches  itself 
to  the  memory  of  that  pleasurable  action,  and  the  connection  with 
the  initial  experience  of  passivity  makes  it  possible — often  only  after 
conscious  and  recollected  effort — to  repress  it  and  replace  it  by  a 
primary  symptom  of  defense.  The  third  period,  that  of  apparent 
healthiness  but  really  of  successful  defense,  begins  with  the  symptoms 
of  scrupulousness,  shame  and  diffidence. 

"The  next  period,  the  disease  is  characterized  by  the  return  of  the 
repressed  reminiscences,  hence,  by  the  failure  of  the  defense;  but  it 
remains  undecided  whether  the  awakening  of  the  same  is  more  fre- 
quently accidental  and  spontaneous,  or  whether  it  appears  in  conse- 
quence of  actual  sexual  disturbances,  that  is,  as  additional  influences 
of  the  same.  But  the  revived  reminiscences  and  the  reproaches  formed 
from  them  never  enter  into  consciousness  unchanged,  but  what  becomes 
conscious  as  an  obsession  and  obsessive  affect  and  substitutes  the 
pathogenic  memory  in  the  conscious  life,  are  compromise  formations 
between  the  repressed  and  the  repressing  ideas. 

"In  order  to  describe  clearly  and  probably  convincingly  the  pro- 
cesses of  repression,  the  return  of  the  repression,  and  the  formation  of 
the  pathological  ideas  of  compromise,  we  would  have  to  decide  upon 
very  definite  hypotheses  concerning  the  substratum  of  the  psychic 
occurrence  and  consciousness.  As  long  as  we  wish  to  avoid  it  we  will 
have  to  rest  content  with  the  following  rather  figuratively  under- 
stood observations.  Depending  on  whether  the  memory  content  of 
the  reproachful  action  alone  forces  an  entrance  into  consciousness  or 
whether  it  takes  with  it  the  accompanying  reproachful  affect,  we  have 
two  forms  of  compulsion  neurosis.  The  first  represents  the  typical 
obsessions,  the  content  of  which  attracts  the  patient's  attention; 
only  an  indefinite  displeasure  is  perceived  as  an  affect,  whereas,  for 
the  content  of  the  obsession  the  only  suitable  affect  would  be  one  of 
reproach.  The  content  of  the  obsession  is  doubly  distorted  when 
compared  to  the  content  of  the  infantile  compulsive  act.  First,  some- 
thing actual  replaces  the  past  experience,  and  second,  the  sexual  is 
substituted  by  an  analogous  non-sexual  experience.  These  two 
changes  are  the  results  of  the  constant  tendency  to  the  repression 
still  in  force  which  we  will  attribute  to  the  'ego.'  The  influence 
of  the  revived  pathogenic  memory  is  shown  by  the  fact  that  the 
content  of  the  obsession  is  still  partially  identical  with  the  repressed, 
or  can  be  traced  to  it  by  a  correct  stream  of  thought.  If,  with  the 
help  of  the  psychoanalytic  method,  we  reconstruct  the  origin  of  one 
individual  obsession  we  find  that  one  actual  impression  instigated 
two  diverse  streams  of  thought,  and  that  the  one  which  passed  over 
the  repressed  memory,  though  incapable  of  consciousness  and  cor- 


608  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

rection,  proves  to  be  just  as  correctly  formed  logically  as  the  other. 
If  the  results  of  the  two  psychic  operations  disagree,  the  contradiction 
between  the  two  may  never  be  brought  to  logical  adjustment,  but  as 
a  compromise  between  the  resistance  and  the  pathological  result 
of  thought  an  apparently  absurd  obsession  enters  into  consciousness 
beside  the  normal  result  of  the  thought.  If  both  streams  of  thought 
yield  the  same  result,  they  reinforce  each  other  so  that  the  normally 
gained  result  of  thought  now  behaves  psychically  like  an  obsession. 
Wherever  neurotic  compulsion  manifests  itself  psychically  it  originates 
from  repression.  The  obsessions  have,  as  it  were,  a  psychical  course 
of  compulsion  which  is  due,  not  to  their  own  validity,  but  to  the  source 
from  which  they  originate,  or  to  the  source  which  furnishes  a  part  of 
their  validity. 

"A  second  form  of  compulsion  neurosis  results  if  the  repressed 
reproach  and  not  the  repressed  content  of  memory  forces  a  replace- 
ment in  the  conscious  psychic  life.  Through  a  psychic  admixture,  the 
affect  of  the  reproach  can  change  itself  into  any  other  affect  of  dis- 
pleasure, and  if  this  occurs  there  is  nothing  to  hinder  the  substituting 
affect  from  becoming  conscious.  Thus  the  reproach  (of  having  per- 
formed in  childhood  some  sexual  actions)  may  be  easily  transformed 
into  shame  (if  some  one  else  becomes  aware  of  it),  into  hypochondriacal 
anxiety  (because  of  the  physical  harmful  consequences  of  those 
reproachful  acts),  into  social  anxiety  (fearing  punishment  from  others), 
into  religious  anxiety,  into  delusions  of  observation  (fear  of  betraying 
those  actions  to  others),  into  fear  of  temptations  (justified  distrust 
in  one's  own  moral  ability  of  resistance),  etc.  Besides,  the  memory 
content  of  the  reproachful  action  may  also  be  represented  in  conscious- 
ness, or  it  may  be  altogether  concealed,  which  makes  the  diagnosis 
very  difficult.  Many  cases  which  on  superficial  examination  are  taken 
as  ordinary  (neurasthenic)  hypochondria  often  belong  to  this  group 
of  compulsive  affects;  the  very  frequently  so-called  'periodic  neuras- 
thenia' or  'periodic  melancholia'  especially  seem  to  be  explained  by 
compulsive  affects  or  obsessions,  a  recognition  not  unimportant 
therapeutically. 

"Beside  these  compromise  symptoms  which  signify  the  return  of 
the  repression  and  hence  a  failure  of  the  originally  achieved  defense, 
the  compulsion  neurosis  forms  a  series  of  other  symptoms  of  a  totally 
different  origin.  The  ego  really  tries  to  defend  itself  against  those 
descendants  of  the  initial  repressed  reminiscence,  and  in  this  conflict 
of  defense  it  produces  symptoms  which  may  be  comprehended  as 
'secondary  defense.'  These  are  throughout  'protective  measures' 
which  have  performed  good  service  in  the  struggle  carried  on  against 
the  obsessions  and  the  obsessing  affects.  If  these  helps  in  the  conflict 
of  the  defense  really  succeed  in  repressing  anew  the  symptoms  of 
return  obtruding  themselves  on  the  ego,  the  compulsion  then  trans- 
mits itself  on  the  protective  measures  themselves  and  produces  a 
third  form  of    the  'compulsion    neurosis,'   the  compulsive  action. 


COMPULSION  NEUROSIS  609 

These  are  never  primary,  they  never  contain  anything  else  but  a 
defense,  never  an  aggression.  Psychic  analysis  shows  that  despite 
their  peculiarity  they  can  always  be  fully  explained  by  reduction  to 
the  compulsive  reminiscence  which  they  oppose. 

''One  example  instead  of  many:  An  eleven-year-old  boy  has 
obsessively  arranged  for  himself  the  following  ceremonial  before 
going  to  bed :  He  could  not  fall  asleep  unless  he  related  to  his  mother 
most  minutely  all  experiences  of  the  day;  not  the  smallest  scrap  of 
paper  or  any  other  rubbish  was  allowed  in  the  evening  on  the  carpet 
of  his  bedroom.  The  bed  had  to  be  moved  close  to  the  wall,  three  chairs 
had  to  stand  in  front  of  it,  and  the  pillows  had  to  lie  in  just  such  a 
position.  In  order  to  fall  asleep  he  had  to  kick  with  both  legs  a  number 
of  times,  and  then  had  to  lie  on  the  side.  This  was  explained  as  fol- 
lows: Years  before,  while  putting  this  pretty  boy  to  sleep,  the  servant 
girl  made  use  of  this  opportunity  to  lay  over  him  and  assault  him 
sexually.  When  this  reminiscence  was  later  awakened  by  a  recent 
experience  it  made  itself  known  to  consciousness  by  the  compulsion 
in  the  above-mentioned  ceremonial  which  sense  could  really  be  sur- 
mised and  the  details  verified  by  psychoanalysis.  The  chairs  before 
the  bed  which  was  close  to  the  wall — so  that  no  one  could  have  access 
to  it;  the  arrangement  of  the  pillows  in  a  definite  manner — so  that 
they  should  be  differently  arranged  than  they  were  on  that  evening; 
the  motion  with  the  legs — to  kick  away  the  person  lying  on  him; 
sleeping  on  the  side — because  during  that  scene  he  lay  on  his  back; 
the  detailed  confession  to  his  mother — because  in  consequence  of  the 
prohibition  of  his  seductress  he  concealed  from  his  mother  this  and 
other  sexual  experiences;  finally,  keeping  the  floor  of  his, bedroom 
clean — because  this  was  the  main  reproach  which  he  had  to  hear 
from  his  mother  up  to  that  time. 

"The  secondary  defense  of  the  obsessions  can  be  brought  about  by 
a  forcible  deviation  to  other  thoughts  of  possibly  contrary  content; 
hence,  in  case  of  success  there  is  a  compulsive  reasoning  regularly, 
concerning  abstract  and  transcendental  subjects,  because  the  repressed 
ideas  always  occupied  themselves  with  the  sensuous.  Or  the  patient 
tries  to  become  master  of  every  compulsive  idea  through  logical  labor 
and  by  appeahng  to  his  conscious  memory;  this  leads  to  compulsive 
thinking  and  examination  to  doubting  mania.  The  priority  of  the 
perception  before  the  memory  in  these  examinations  at  first  induce 
and  then  force  the  patient  to  collect  and  preserve  all  objects  with 
which  he  comes  in  contact.  The  secondary  defense  against  the  com- 
pulsive affects  results  in  a  greater  number  of  defensive  measures  which 
are  capable  of  being  transformed  into  compulsive  actions.  These  can 
be  grouped  according  to  their  tendency.  We  may  have  measures  of 
penitence  (irksome  ceremonial  and  observation  of  numbers),  of  pre- 
vention (diverse  phobias,  superstition,  pedantry,  aggravation  of  the 
primary  symptom  of  scrupulousness),  measures  of  fear  of  betrayal 
(collecting  papers  and  shyness),  and  measures  of  becoming  uncon- 
39 


610  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

scious  (dipsomania).  Among  these  compulsive  acts  and  impulses 
the  phobias  play  the  greatest  part  as  limitations  of  the  patient's 
existence. 

"There  are  cases  in  which  we  can  observe  how  the  compulsion 
becomes  transferred  from  the  idea  or  affect  to  the  measure,  and 
other  cases  in  which  the  compulsion  ocillates  between  the  returning 
symptoms  of  secondary  defense.  But  there  are  also  cases  in  which 
no  obsessions  are  really  formed,  but  the  repressed  reminiscence  imme- 
diately becomes  replaced  by  the  apparent  primary  defensive  measure. 
Here  that  stage  is  attained  at  a  bound  which  otherwise  ends  the 
course  of  the  compulsion  neurosis  only  after  the  conflict  of  the  defense. 
Grave  cases  of  this  affection  end  either  with  a  fixation  of  ceremonial 
actions,  general  doubting  mania,  or  in  an  existence  of  eccentricity 
conditioned  by  phobias. 

"That  the  obsessions  and  everything  derived  from  them  are  not 
believed  is  probably  due  to  the  fact  that  the  defense  symptom  of 
scrupulousness  was  formed  during  the  first  repression  and  gained 
compulsive  validity.  The  certainty  of  having  lived  morally  through- 
out the  whole  period  of  the  successful  defense  makes  it  impossible 
to  give  credence  to  the  reproach  which  the  obsession  really  involves. 
Only  transitorily  during  the  appearance  of  a  new  obsession,  and  now 
and  then  in  melancholic  exhaustive  states  of  the  ego  do  the  morbid 
symptoms  of  the  return  also  enforce  the  belief.  The  'compulsion'  of 
the  psychic  formations  here  described  has  in  general  nothing  to  do 
with  the  recognition  through  belief,  and  is  not  to  be  mistaken  for  that 
moment  which  is  designated  as  'strength'  or  'intensity'  of  an  idea. 
Its  main  characteristic  lies  in  its  inexplicableness  through  psychic 
activities  of  conscious  ability,  and  this  character  undergoes  no  change 
whether  the  idea  to  which  the  compulsion  is  attached  is  stronger  or 
weaker,  more  or  less  intensively '  elucidated,' '  supplied  with  energy,'  etc. 

"  The  reason  for  the  unassailableness  of  the  obsession  or  its  deriva- 
tive is  due  only  to  its  connection  with  the  repressed  memory  of  early 
childhood,  for  as  soon  as  we  succeed  in  making  it  conscious,  for  which 
the  psychotherapeutic  methods  already  seem  quite  sufficient,  the  com- 
pulsion, too,  becomes  detached." 

The  mechanism  of  the  compulsion  neurosis  is  therefore  seen  to  be 
an  extremely  complicated  one  and  one  which  produces  a  great  variety 
of  symptoms,  with  all  possible  ramifications  of  meaning. 

This  mechanism  as  set  forth  in  this  rather  intricate  statement  by 
Freud  may  be  more  simply  stated  by  saying  that,  in  distinction 
from  hysteria  in  which  the  disguise  is  brought  about  by  a  transfer 
of  the  repressed  material  into  symbols  of  bodily  ailment- — conversion — 
in  the  compulsion  neurosis  the  disfigurement  is  kept  wholly  within 
recognized  psychological  territory.  The  distortion  is  produced  by 
displacement  of  the  affect  upon  indifferent  ideas  and  the  development 
of  a  purificatory  ceremonial.  Fear  of  animals  (snakes,  mice,  etc.) 
may  be  the  accepted  conscious  equivalent  of  fear  of  sexuality  with 


COMPULSION  NEUROSIS— SYMPTOMS  611 

a  type  of  ceremonial,  and  is  well  illustrated  in  the  case  of  the  eleven- 
year-old  boy  cited. 

It  is  important  to  bear  in  mind  that  the  theory  of  infantile  sexual 
trauma  has  long  since  been  discarded  by  Freud  himself.  More  atten- 
tion is  being  paid  to  the  present  difficulties  of  the  patient,  particu- 
larly under  the  influence  of  Jung,  who  would  always  ask  what  task  does 
the  patient  wish  to  avoid?  The  neurotic  way  of  avoiding  a  task  or 
duty  is  to  revert  to  infantile  methods.  The  infantile  experiences  do 
not  explain  the  difficulty  but  only  the  symptoms.  They  only  show 
why  certain  symptoms  are  used,  because  they  represent  material  in 
the  life  of  the  patient  and  are  merely  being  brought  in  this  stage  to 
serve  a  purpose. 

Symptoms. — The  symptoms  of  the  compulsive  neurosis  are  very 
varied.  They  have  to  do  with  all  types  of  obsessional  thinking  and 
acting,  that  is,  thinking  and  acting  which  takes  place  aside  from  the 
volition  of  the  patient,  which  he  cannot  prevent  but  which  he  must 
yield  to,  as  already  described.  This  is  the  compulsion  element  which 
gives  the  name  to  the  neurosis. 

^  The  symptoms  have  been  variously  divided  and  may  be  described 
under  the  form  of  motor  symptoms,  obsessive  acts  of  various  sorts; 
tics,  spasmodic  torticollis,  even  epileptic  attacks;  sensory  symptoms, 
obsessive  sensations,  amounting  at  times  to  well-marked  hallucina- 
tions; affective  symptoms,  obsessive  emotions,  more  particularly 
those  of  doubt  and  fear;  and  ideational  symptoms,  obsessive  ideas, 
such  as  continual  questioning. 

The  commonest  and  best  known  of  the  obsessions  are  the  phobias 
or  fears  which  usually  refer  to  some  very  specific  object  or  set  of 
conditions  which  acquire  their  quality  of  fear  as  the  result  of  taking 
.over  an  affect  by  displacement  which  is  of  deep  though  unconscious 
significance  to  the  patient.  Thus  there  are  misopJiobia  (fear  of  dirt 
or  contamination),  metallophohia  (fear  of  metal,  door-knobs,  money, 
etc.),  agoraphobia  (fear  of  wide  or  open  spaces),  claustrophobia  (fear  of 
narrow  or  closed  spaces),  pyrophobia  (fear  of  fire)  and  so  on  indefinitely. 

The  obsessions  of  doubt — folie  de  doute — are  common  and  result 
in  a  state  of  mind  in  which  the  patient  is  torn  between  two  courses  of 
conduct  and  cannot  choose,  or  having  done  something,  such  as  turn 
out  the  gas  before  going  to  bed,  is  seized  with  a  doubt  as  to  whether 
he  really  did  do  it  or  not  and  must  get  up  and  satisfy  himself.  Then 
doubts  when  they  refer  to  religious  or  philosophical  matters  lead  to 
continuing  questionings  and  elaborate  processes  of  reasoning  from 
which  the  patient  cannot  free  his  mind. 

Quite  allied  to  the  phobias  and  doubts  are  certain  moral  obsessions 
such  as  overconscientiousness  and  exaggerated  scrupulosity. 

Of  the  various  obsessional  activities  the  so-called  manias  are  best 
known.  Thus  there  are  kleptomania  (a  compulsion  to  steal),  pyro- 
mania  (a  compulsion  to  set  something  on  fire),  dipsomania  (a  com- 
pulsion to  drink),  etc. 


G12  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

In  addition  there  are  all  sorts  of  less  easily  classified  and  more 
complex  forms  of  obsessional  ways  of  thinking,  feeling  and  acting. 
There  are  complicated  ceremonials,  such  as  that  of  the  eleven-year- 
old  boy  already  cited,  ways  of  arranging  things  that  must  be  carried 
out.  Strange,  and  to  the  patient  unexplicable,  attractions  and  repul- 
sions, dislikes  for  certain  kinds  of  food,  or  persons  with  a  particular 
color  of  hair,  all  manner  of  tics,  habits,  mannerisms,  ceremonials, 
the  necessity  for  touching  things — delire  de  toucher — fixed  ideas, 
hypochondrias,  etc. 

Such  psychological  phenomena  must,  of  course,  have  a  reason  for 
their  existence,  and  if  the  reason  is  not  apparent  it  cannot  be  explained 
by  the  patient;  it  must  be  sought  by  psychoanalysis  in  the  unconscious. 

These  obsessions  produce  a  tremendous  amount  of  mental  unrest 
and  suffering  if  they  are  not  yielded  to,  and  a  sense  of  relief  is  expe- 
rienced when  they  are  yielded  to,  oftentimes,  however,  with  a  following 
sense  of  remorse  for  having  yielded. 

The  compulsion  ideas  and  acts  represent  compromise  formations 
which  permit  the  patient  to  obtain  satisfactions  in  infantile  ways,  i.  e., 
to  revert  to  old  ways  of  gaining  pleasure  which  were  active  and 
important  in  infanc}^  when  the  erogenous  zones  were  as  yet  not 
clearly  difi^erentiated.  Here  we  find  the  explanation  for  urinary 
and  fecal  phantasies,  for  certain  cutaneous,  anal,  and  gastro-intestinal 
satisfactions  which  are  used  as  ways  of  getting  pleasure  when  driven 
away  from  reality.  They  become  infantile  ways  of  reacting  to  reality 
situations  and  so  are  inefficient,  sick  ways. 

Anxiety  Hysteria. — Anxiety  hysteria,  as  the  name  indicates,  occupies 
a  midposition  between  conversion  hysteria  on  the  one  hand  and 
anxiety  neurosis  on  the  other.  There  is,  so  to  speak,  a  combination 
of  the  two  conditions,  although  this  is  not  quite  the  situation.  In 
conversion  hysteria  the  affect  of  the  repressed  complexes  is  drafted 
into  bodily  innervation  and  produces  the  physical  symptoms  of  the 
disease.  In  anxiety  hysteria  the  affect  remains  in  the  mental  sphere, 
producing  there  various  phobias.  In  anxiety  neurosis,  as  will  be 
seen  later,  the  anxiety  has  its  origin  not  at  the  psychical,  but  at  the 
physiological  level  and  is  a  representation  in  the  psychic  sphere  of  a 
disturbance  in  the  somatic.  In  anxiety  hysteria,  the  anxiety  is  also 
produced  at  the  physiological  level,  but  it  is  a  secondary  symptom  and 
is  the  result  of  the  physiological  accompaniments  of  the  emotions 
which  go  with  the  phobias,  such  as  difficulty  of  breathing,  cardiac 
palpitation,  etc. 

Anxiety  hysteria  is  one  of  the  most  widel}'  distributed  diseases. 
It  is  particularly  the  disease  which  manifests  itself  in  childhood  and 
from  which  most  of  the  so-called  nervous  children  suffer.  It  is  much 
more  easy  of  approach  therapeutically  than  the  compulsion  neurosis, 
its  accessibility  being  comparable  to  that  of  hysteria,  and  so  offers 
greater  opportunities  for  treatment.  Probably  many  cases  of  this 
disorder  are  included  under  Janet's  psychasthenia. 


ANXIETY  NEUROSIS  61^ 


THE  ACTUAL  NEUROSES. 


Anxiety  Neurosis. — The  anxiety  neurosis  was  separated  from  the 
general  group  of  actual  neuroses  and  psychoneuroses  by  Freud.  The 
name  anxiety  neurosis  indicates  that  the  symptoms  all  group  them- 
selves about  the  cardinal  symptom  of  anxiety,  and  it  is  significant 
of  this  anxiety,  that,  while  it  is  a  psychic  fact,  it  is  still  not  of 
psychic  but  of  somatic  origin.  The  anxiety,  therefore,  is  not  suscep- 
tible of  being  analyzed  into  psychic  components,  but  its  source  can 
only  be  found  at  the  physiological  level.  This  anxiety  arising  at  the 
physiological  level  and  manifesting  itself  in  the  psychic  sphere  then 
becomes  a  "free-floating  anxiety"  which  may  attach  itself  to  any 
idea  and  therefore  appear  to  be  of  psychic  origin.  On  the  other  hand 
it  may  express  itself  simply  as  anxiety  without  ideational  content. 

Anxiety  may  thus  be  seen  to  be  the  correlative  of  fear.  While  fear 
is  the  emotion  which  corresponds  to  a  danger  threatening  the  organism 
from  outside,  anxiety  corresponds  to  a  danger  which  threatens  the 
organism  from  within. 

It  will  help  to  make  understandable  what  has  to  be  said  about  the 
anxiety  neurosis  if  it  is  understood  at  the  start  that  the  whole  sex 
relationship  which  is  consummated  by  the  sexual  act  consists  of  two 
parts,  a  somatic  and  a  psychic.  In  contrast  to  neurasthenia,  which 
results  when  the  discharge  of  energy  is  inadequate  upon  the  somatic 
side,  anxiety  neurosis  occurs  whenever  the  discharge  is  inadequate 
in  the  psychic  sphere. 

Symptoms. — The  following  is  the  description  of  the  symptomatology 
of  anxiety  neurosis  as  given  by  Freud  :^ 

"1.  General  Irritability. — This  is  a  frequent  nervous  symptom, 
common  as  such  to  many  nervous  states.  It  is  mentioned  here 
because  it  constantly  occurs  in  the  anxiety  neurosis  and  is  of  theoret- 
ical significance.  Increased  irritability  always  points  to  an  accu- 
mulation of  excitement  or  to  an  inability  to  bear  accumulation,  hence 
to  an  absolute  or  relative  accumulation  of  excitement.  The  expression 
of  this  increased  irritability  through  an  auditory  hyperesthesia  is 
especially  worth  mentioning;  it  is  an  oversensitiveness  for  noises, 
which  symptom  is  certainly  to  be  explained  by  the  congenital  intimate 
relationship  between  auditory  impressions  and  fright.  Auditory 
hyperesthesia  is  frequently  found  as  a  cause  of  insomnia,  of  which 
more  than  one  form  belongs  to  anxiety  neurosis. 

"2.  Anxious  Expectation. — I  cannot  better  explain  the  condition 
that  I  have  in  mind  than  by  this  name  and  by  some  appended 
examples.  A  woman,  for  example,  who  suffers  from  anxious  expec- 
tation thinks  of  influenza-pneumonia  whenever  her  husband,  who  is 
aflflicted  with  a  catarrhal  condition,  has  a  coughing  spell;  and  in  her 
mind  she  sees  a  passing  funeral  procession.    If  on  her  way  home  she 

1  Loc.  cit. 


614         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

sees  two  persons  standing  together  in  front  of  her  house  she  cannot 
refrain  from  the  thought  that  one  of  her  children  fell  out  of  the  window; 
if  she  hears  the  bell  ring  she  thinks  that  someone  is  bringing  her 
mournful  tidings,  etc. ;  yet  in  none  of  these  cases  is  there  any  special 
reason  for  exaggerating  a  mere  possibility. 

"The  anxious  expectation  naturally  reflects  itself  constantly  in 
the  normal,  and  embraces  all  that  is  designated  as  'uneasiness  and 
a  tendency  to  a  pessimistic  conception  of  things/  but  as  often  as 
possible  it  goes  beyond  such  a  plausible  uneasiness,  and  it  is  frequently 
recognized  as  a  part  of  constraint  even  by  the  patient  himself. 
For  one  form  of  anxious  expectation,  namely,  that  which  refers  to 
one's  own  health,  we  can  reserve  the  old  name  of  hypochondria. 
Hypochondria  does  not  always  run  parallel  with  the  height  of  the 
general  anxious  expectation;  as  a  preliminary  stipulation  it  requires 
the  existence  of  paresthesias  and  annoying  somatic  sensations.  Hypo- 
chondria is  thus  the  form  preferred  by  the  genuine  neurasthenics 
whenever  they  merge  into  the  anxiety  neurosis,  a  thing  which 
frequently  happens. 

"As  a  further  manifestation  of  anxious  expectation  we  may  men- 
tion the  frequent  tendency  observed  in  morally  sensitive  persons  to 
pangs  of  conscience,  scrupulosity,  and  pedantry,  which  varies,  as  it 
were,  from  the  normal  to  its  aggravation  as  doubting  mania. 

"Anxious  expectation  is  the  most  essential  symptom  of  the  neurosis; 
it  also  clearly  shows  a  part  of  its  theory.  It  can  perhaps  be  said  that 
we  have  here  a  quantum  of  freely  floating  anxiety  which  controls  the 
choice  of  ideas  by  expectation  and  is  forever  ready  to  unite  itself 
with  any  suitable  ideation. 

"3.  This  is  not  the  only  way  in  which  the  anxiousness,  usually 
latent  but  constantly  lurking  in  consciousness,  can  manifest  itself. 
On  the  contrary  it  can  also  suddenly  break  into  consciousness  with- 
out being  aroused  by  the  issue  of  an  idea,  and  thus  provoke  an  attack 
of  anxiety.  Such  an  attack  of  anxiety  consists  of  either  the  anxious 
feeling  alone  without  any  associated  idea,  or  of  the  nearest  interpreta- 
tion of  the  termination  of  life,  such  as  the  idea  of  'sudden  death'  or 
threatening  insanity;  or  the  feeling  of  anxiety  becomes  mixed  with 
some  paresthesia  (similar  to  the  hysterical  aura) ;  or  finally  the  anxious 
feeling  may  be  combined  with  a  disturbance  of  one  or  many  somatic 
functions,  such  as  respiration,  cardiac  activity,  the  vasomotor  inner- 
vation, and  the  glandular  activity.  From  this  combination  the 
patient  renders  especially  prominent  now  this  and  now  the  other 
moment.  He  complains  of  'heart  spasms,'  'heavy  breathing,'  'profuse 
perspiration,'  'inordinate  appetite,'  etc.,  and  in  his  description  the 
feeling  of  anxiety  is  put  to  the  background  or  it  is  rather  vaguely 
described  as  'feeling  badly,'  'uncomfortably,'  etc. 

"4.  What  is  interesting  and  of  diagnostic  significance  is  the  fact 
that  the  amount  of  admixture  of  these  elements  in  the  attack  of 
anxiety  varies  extraordinarily,  and  that  almost  any  accompanying 


ANXIETY  NEUROSIS  615 

symptom  can  alone  constitute  the  attack  as  well  as  the  anxiety  itself. 
Accordingly  there  are  rudimentary  attacks  of  anxiety,  and  equivalents 
for  the  attack  of  anxiety,  probably  all  of  equal  significance  in  showing 
a  profuse  and  hitherto  little-appreciated  richness  in  forms.  A  more 
thorough  study  of  these  larvated  states  of  anxiety  (Hecker)  and  their 
diagnostic  division  from  other  attacks  ought  soon  to  become  the 
necessary  work  for  the  neuropathologist. 

"I  now  add  a  list  of  those  forms  of  attacks  of  anxiety  with  which 
I  am  acquainted.    There  are  attacks: 

"  (a)  With  disturbances  of  heart  action,  such  as  palpitation  with 
transitory  arrythmia,  with  longer-continued  tachycardia  up  to  grave 
states  of  heart  weakness,  the  differentiation  of  which  from  organic 
heart  affection  is  not  always  easy;  among  such  we  have  the  pseudo- 
angina  pectoris,  a  delicate  diagnostic  sphere. 

"(6)  With  disturbances  of  respiration,  many  forms  of  nervous 
dyspnea,  asthma-like  attacks,  etc.  I  assert  that  even  these  attacks 
are  not  always  accompanied  by  conscious  anxiety; 

"  (c)  Of  profuse  perspiration,  often  nocturnal ; 

"  (d)  Of  trembling  and  shaking  which  may  readily  be  mistaken  for 
hysterical  attacks; 

"  (e)  Of  inordinate  appetite,  often  combined  with  dizziness; 

"  (/)  Of  attack-like  appearing  diarrhea; 

"  (g)  Of  locomotor  dizziness; 

"  (h)  Of  so-called  congestions,  embracing  all  that  was  called  vaso- 
motor neurasthenia;  and 

"  (i)  Of  paresthesias  (these  are  seldom  without  anxiety  or  a  similar 
discomfort) . 

"5.  Very  frequently  the  nocturnal  frights  (pavor  nocturnus  of 
adults)  usually  combined  with  anxiety,  dyspnea,  perspiration,  etc., 
is  nothing  other  than  a  variety  of  the  attack  of  anxiety.  This  dis- 
turbance determines  a  second  form  of  insomnia  in  the  sphere  of 
the  anxiety  neurosis.  Moreover,  I  became  convinced  that  even  the 
pavor  nocturnus  of  children  evinces  a  form  belonging  to  the  anxiety 
neurosis.  The  hysterical  tinge  and  the  connection  of  the  fear  with 
the  reproduction  of  appropriate  experience  or  dream,  makes  the  pavor 
nocturnus  of  children  appear  as  something  peculiar,  but  it  also  occurs 
alone  without  a  dream  or  a  recurring  hallucination. 

"6.  'Vertigo.' — This  in  its  lightest  forms  is  better  designated  as 
'dizziness,'  assumes  a  prominent  place  in  the  group  of  symptoms  of 
anxiety  neurosis.  In  its  severer  forms  the  'attack  of  vertigo,'  with 
or  without  fear,  belongs  to  the  gravest  symptoms  of  the  neurosis. 
The  vertigo  of  the  anxiety  neurosis  is  neither  a  rotatory  dizziness  nor 
is  it  confined  to  certain  planes  or  lines  like  Meniere's  vertigo.  It  belongs 
to  the  locomotor  or  coordinating  vertigo,  like  the  vertigo  in  paralysis 
of  the  ocular  muscles;  it  consists  in  a  specific  feeling  of  discomfort 
which  is  accompanied  by  sensations  of  a  heaving  ground,  sinking  legs, 
of  the  impossibility  to  continue  in  an  upright  position,  and  at  the 


616  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

same  time  there  is  a  feeling  that  the  legs  are  as  heavy  as  lead,  they 
shake,  or  give  way.  This  vertigo  never  leads  to  falling.  On  the  other 
hand,  I  would  like  to  state  that  such  an  attack  of  vertigo  may  also 
be  substituted  by  a  profound  attack  of  syncope.  Other  fainting-like 
states  in  the  anxiety  neurosis  seem  to  depend  on  a  cardiac  collapse. 

"The  vertigo  attack  is  frequently  accompanied  by  the  worst  kind 
of  anxiety  and  is  often  combined  with  cardiac  and  respiratory  dis- 
turbances. Vertigo  of  elevations,  mountains  and  precipices,  can  also 
be  frequently  observed  in  anxiety  neurosis;  moreover,  I  do  not  know 
whether  we  are  still  justified  in  recognizing  a  vertigo  of  stomachic  origin. 

"7.  On  the  basis  of  the  chronic  anxiousness  (anxious  expectation) 
on  the  one  hand,  and  the  tendency  to  vertiginous  attacks  of  anxiety 
on  the  other,  there  develop  two  groups  of  typical  phobias;  the  first 
refers  to  the  general  physiological  menaces,  while  the  second  refers 
to  locomotion.  To  the  first  group  belong  the  fear  for  snakes,  thunder- 
storms, darkness,  vermin,  etc.,  as  well  as  the  typical  moral  over- 
scrupulousness,  and  the  forms  of  doubting  mania.  Here  the  available 
fear  is  merely  used  to  strengthen  those  aversions  which  are  instinctively 
implanted  in  every  man.  But  usually  a  compulsively  acting  phobia 
is  formed  only  after  a  reminiscence  is  added  to  an  experience  in  which 
this  fear  could  manifest  itself;  as,  for  example,  after  the  patient  has 
experienced  a  storm  in  the  open  air.  To  attempt  to  explain  such 
cases  as  mere  continuations  of  strong  impressions  is  incorrect.  What 
makes  these  experiences  significant  and  their  reminiscences  durable 
is  after  all  only  the  fear  which  could  at  that  time  appear  and  can  also 
appear  today.  In  other  words  such  impressions  remain  forceful  only 
in  persons  with  'anxious  expectations.' 

"The  other  group  contains  agoraphobia  with  all  its  accessory 
forms,  all  of  which  are  characterized  by  their  relation  to  locomotion. 
As  a  determination  of  the  phobia  we  frequently  find  a  precedent  attack 
of  vertigo;  I  do  not  think  that  it  can  always  be  postulated.  Occa- 
sionally, after  a  first  attack  of  vertigo  without  fear,  we  see  that  though 
locomotion  is  always  accompanied  by  the  sensation  of  vertigo,  it 
remains  possible  without  any  restrictions,  but  as  soon  as  fear  attaches 
itself  to  the  attack  of  vertigo,  locomotion  fails,  under  the  conditions 
of  being  alone,  narrow  streets,  etc. 

"The  relation  of  these  phobias  to  the  phobias  of  obsessions,  which 
mechanism  I  discussed  above,^  is  as  follows:  The  agreement  lies  in 
the  fact  that  here  as  there,  an  idea  becomes  obsessive  through  its 
connection  with  an  available  affect.  The  mechanism  of  transposition 
of  the  affect  therefore  holds  true  for  both  kinds  of  phobias.  But  in 
phobias  of  the  anxiety  neurosis  this  affect  is  (1)  a  monotonous  one, 
it  is  always  one  of  anxiety;  (2)  it  does  not  originate  from  a  repressed 
idea,  and  on  psychological  analysis  it  proves  itself  not  further  reducible, 
nor  can  it  be  attacked  through  psychotherapy.    The  mechanism  of 

1  Die  Abwehr-Neuropsy chosen,  Neurol.  Centralbl.,  1894,  No.  10,  u.  ii. 


ANXIETY  NEUROSIS  617 

substitution  does  not  therefore  hold  true  for  the  phobias  of  anxiety 
neurosis. 

"Both  kinds  of  phobias  (or  obsessions)  often  occur  side  by  side, 
though  the  atypical  phobias  which  depend  on  obsessions  need  not 
necessarily  develop  on  the  basis  of  anxiety  neurosis.  A  very  frequent, 
ostensibly  complicated  mechanism  appears  if  the  content  of  an 
original  simple  phobia  of  anxiety  neurosis  is  substituted  by  another 
idea,  the  substitution  is  then  subsequently  added  to  the  phobia.  The 
'protective  measures'  originally  employed  in  combating  the  phobia 
are  most  frequently  used  as  substitutions.  Thus,  for  example,  from 
the  effort  to  provide  one's  self  with  counter-evidence  that  one  is  not 
crazy,  contrary  to  the  assertion  of  the  hypochondriacal  phobia,  there 
results  a  reasoning  mania.  The  hesitations,  doubts,  and  the  many 
repetitions  of  the  folie  du  doute  originate  from  the  justified  doubt 
concerning  the  certainty  of  one's  own  stream  of  thoughts,  for,  through 
the  compulsive-hke  idea  one  is  surely  conscious  of  so  obstinate  a 
disturbance,  etc.  It  may  therefore  be  claimed  that  many  syndromes 
of  compulsion  neurosis,  like  folie  de  doute  and  similar  ones,  can  clinic- 
ally, if  not  notionally,  be  attributed  to  anxiety  neurosis.^ 

"8.  The  digestive  functions  in  anxiety  neurosis  are  subject  to  very 
few  but  characteristic  disturbances.  Sensations  like  nausea  and 
sickly  feeling  are  not  rare,  and  the  symptom  of  inordinate  appetite 
alone  or  with  other  congestions,  may  serve  as  a  rudimentary  attack 
of  anxiety.  As  a  chronic  alteration  analogous  to  the  anxious  expecta- 
tions one  finds  a  tendency  to  diarrhea  which  has  occasioned  the 
queerest  diagnostic  mistakes.  If  I  am  not  mistaken  it  is  this  diarrhea 
to  which  Moebius^  has  recently  called  attention  in  a  small  article. 
I  believe,  moreover,  that  Peyer's^  reflex  diarrhea  which  he  attributes 
to  a  disease  of  the  prostate  is  nothing  other  than  the  diarrhea  of  anxiety 
neurosis.  The  deceptive  reflex  relation  is  due  to  the  fact  that  the  same 
factors  which  are  active  in  the  origin  of  such  prostatic  affections  also 
come  into  play  in  the  etiology  of  anxiety  neurosis. 

"The  behavior  of  the  gastro-intestinal  function  in  anxiety  neurosis 
shows  a  sharp  contrast  to  the  influence  of  this  same  function  in  neuras- 
thenia. Mixed  cases  often  show  the  familiar  'fluctuations  between 
diarrhea  and  constipation.'  The  desire  to  urinate  in  anxiety  neurosis 
is  analogous  to  the  diarrhea. 

"9.  The  paresthesias  which  accompany  the  attack  of  vertigo  or 
anxiety  are  interesting  because  they  associate  themselves  into  a  firm 
sequence,  similar  to  the  sensations  of  the  hysterical  aura.  But  in 
contrast  to  the  hysterical  aura  I  find  these  associated  sensations 
atypical  and  changeable.  Another  similarity  to  hysteria  is  shown  by 
the  fact  that  in  anxiety  neurosis  a  kind  of  conversion*  into  bodily 

'  Obsessions  et  phobies,  Revue  neurologique,  1895. 

^  Neuropathologische  Beitrage,   1894,  ii,  Heft. 

3  Die  nervosen  Affektionen  des  Darmes,  Wiener  Klinik,  1893. 

*  Freud,  Abwehr-Neuropsychosen. 


618  THE  PSYCHONEVROSES  AND  ACTUAL  NEUROSES 

sensations,  as,  for  example,  into  rheumatic  muscles,  takes  place  which 
otherwise  can  be  overlooked  at  one's  pleasure.  A  large  number  of 
so-called  rheumatics,  who  are  moreover  demonstrable  as  such,  really 
suffer  from  an  anxiety  neurosis.  Besides  this  aggravation  of  the  sen- 
sation of  pain  I  have  observed  in  a  number  of  cases  of  anxiety  neurosis 
a  tendency  toward  hallucinations  which  could  not  be  explained  as 
hysterical. 

"10.  Many  of  the  so-called  symptoms  which  accompany  or  sub- 
stitute the  attack  of  anxiety  also  appear  in  a  chronic  manner.  They 
are  then  still  less  discernible,  for  the  anxious  feeling  accompanying 
them  appears  more  indistinct  than  in  the  attack  of  anxiety.  This 
especially  holds  true  for  the  diarrhea,  vertigo,  and  paresthesias.  Just 
as  the  attack  of  vertigo  can  be  substituted  by  an  attack  of  syncope, 
so  can  the  chronic  vertigo  be  substituted  by  the  continuous  feeling  of 
feebleness,  lassitude,  etc." 

The  Etiology  and  Occurrence  of  Anxiety  Neurosis. — The  following 
remarks  on  the  etiology  and  occurrences  of  anxiety  neurosis  are 
quoted  from  Freud's  original  paper  :^ 

"  In  some  cases  of  anxiety  neurosis  no  etiology  can  readily  be  ascer- 
tained. It  is  noteworthy  that  in  such  cases  it  is  seldom  difficult  to 
demonstrate  a  marked  hereditary  taint. 

"  Where  we  have  reason  to  assume  that  the  neurosis  is  acquired  we 
can  find  by  careful  and  laborious  examination  that  the  etiologically 
effective  moments  are  based  on  a  series  of  injuries  and  influences  from 
the  sexual  life.  These  at  first  appear  to  be  of  a  varied  nature  but 
easily  displayed  the  common  character  which  explains  their  homo- 
geneous effect  on  the  nervous  system.  They  are  found  either  alone 
or  with  other  banal  injuries  to  which  a  reinforcing  effect  can  be  attrib- 
uted. This  sexual  etiology  of  anxiety  neurosis  can  be  demonstrated  so 
preponderately  often  that  I  venture  for  the  purpose  of  this  brief  com- 
munication to  set  aside  all  cases  of  a  doubtful  or  different  etiology. 

"For  the  more  precise  description  of  the  etiological  determinations 
under  which  anxiety  neurosis  occurs,  it  will  be  advisable  to  treat 
separately  those  occurring  in  men  and  those  occurring  in  women. 
Anxiety  neurosis  appears  in  women — disregarding  their  predisposition 
— in  the  following  cases : 

"  (a)  As  virginal  fear  or  anxiety  in  adults.  A  number  of  unequivocal 
observations  showed  me  that  an  anxiety  neurosis,  which  is  almost 
typically  combined  with  hysteria,  can  be  evoked  in  maturing  girls, 
at  the  first  encounter  with  the  sexual  problem,  that  is,  at  the  sudden 
revelation  of  the  things  hitherto  veiled,  by  either  seeing  the  sexual  act, 
or  by  hearing  or  reading  something  of  that  nature; 

"  (6)  As  fear  in  the  newly  married.  Young  women  who  remain 
anesthetic  during  the  first  cohabitation  not  seldom  merge  into  an 
anxiety  neurosis  .which  disappears  after  the  anesthesia  is  displaced  by 

1  Loc.  cit. 


ANXIETY  NEUROSIS  619 

the  normal  sensation.  As  most  young  women  remain  undisturbed 
through  such  a  beginning  anesthesia,  the  production  of  this  fear  requires 
determinants  which  I  will  mention; 

"  (c)  As  fear  in  women  whose  husbands  suffer  from  ejaculatio  precox 
or  from  diminished  potency;  and, 

"  (d)  In  those  whose  husbands  practice  coitus  interruptus  or  reser- 
vatus.  These  cases  go  together,  for  on  analyzing  a  large  number  of 
examples  one  can  easily  be  convinced  that  they  only  depend  on  whether 
the  woman  attained  gratification  during  coitus  or  not.  In  the  latter 
case  one  finds  the  determinant  for  the  origin  of  anxiety  neurosis. 
On  the  other  hand,  the  woman  is  spared  from  the  neurosis  if  the  hus- 
band afflicted  by  ejaculatio  precox  can  repeat  the  congress  with  better 
results  immediately  thereafter.  The  congressu  reservatus  by  means 
of  the  condom  is  not  injurious  to  the  woman  if  she  is  quickly  excited 
and  the  husband  is  very  potent;  in  other  cases  the  noxiousness  of  this 
kind  of  preventive  measure  is  not  inferior  to  the  others.  Coitus 
interruptus  is  almost  regularly  injurious;  but  for  the  woman  it  is 
injurious  only  if  the  husband  practises  it  regardlessly,  that  is,  if  he 
interrupts  coitus  as  soon  as  he  comes  near  ejaculating  without  con- 
cerning himself  about  the  determination  of  the  excitement  of  his 
wife.  On  the  other  hand  if  the  husband  waits  until  his  wife  is  gratified, 
the  coitus  has  the  same  significance  for  the  latter  as  a  normal  one; 
but  then  the  husband  becomes  afflicted  with  an  anxiety  neurosis. 
I  have  collected  and  analyzed  a  number  of  cases  which  furnished 
the  material  for  the  above  statements. 

"  (e)  As  fear  in  widows  and  intentional  abstainers,  not  seldom  in 
typical  combination  with  obsessions;  and 

"  (J)  As  fear  in  the  climacterium  during  the  last  marked  enhance- 
ment of  the  sexual  desire. 

"The  cases  (c),  (d),  and  (e)  contain  the  determinants  under  which 
the  anxiety  neurosis  originates  in  the  female  sex  most  frequently  and 
most  independently,  of  hereditary  predisposition.  I  will  endeavor  to 
demonstrate  in  these — curable,  acquired — cases  of  anxiety  neurosis 
that  the  discovered  sexual  injuries  really  represent  the  etiological 
moments  of  the  neurosis.  But  before  proceeding  I  will  mention  the 
sexual  determinants  of  anxiety  neurosis  in  men.  I  would  like  to 
formulate  the  following  groups,  everyone  of  which  finds  its  analogy 
in  women: 

"  (a)  Fear  of  the  intentional  abstainers;  this  is  frequently  combined 
with  symptoms  of  defense  (obsessions,  hysteria).  The  motives  which 
are  decisive  for  intentional  abstinence  carry  along  with  them  the  fact 
that  a  number  of  hereditarily  burdened  eccentrics,  etc.,  belong  to  this 
category. 

"  (b)  Fear  in  men  with  frustrated  excitement  (during  the  engagement 
period),  persons  who  out  of  fear  for  the  consequences  of  sexual  rela- 
tions satisfy  themselves  with  handling  or  looking  at  the  womap. 
This  group  of  determinants  which  can  moreover  be  transferred  to 


620  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

the  other  sex — -engagement  periods,  relations  with  sexual  forbearance 
— furnish  the  purest  cases  of  the  neurosis. 

"  (c)  Fear  in  men  who  practice  coitus  interruptus.  As  observed 
above,  coitus  interruptus  injures  the  woman  if  it  is  practised  regard- 
less of  the  woman's  gratification;  it  becomes  injurious  to  the  man  if 
in  order  to  bring  about  the  gratification  in  the  woman  he  voluntarily 
controls  the  coitus  by  delaying  the  ejaculation.  In  this  manner  we 
can  understand  why  it  is  that  in  couples  who  practise  coitus  interruptus 
it  is  usually  only  one  of  them  who  becomes  afflicted.  Moreover,  the 
coitus  interruptus  only  rarely  produces  in  man  a  pure  anxiety  neurosis, 
usually  it  is  a  mixture  of  the  same  with  neurasthenia. 

"  (d)  Fear  in  men  in  the  senium.  There  are  men  who  show  a  climac- 
terium like  women,  and  merge  into  an  anxiety  neurosis  at  the  time 
when  their  potency  diminishes  and  their  libido  increases. 

"Finally  I  must  add  two  more  cases  holding  true  for  both  sexes: 

"  (e)  Neurasthenics  merge  into  anxiety  neurosis  in  consequence  of 
masturbation  as  soon  as  they  refrain  from  this  manner  of  sexual  grati- 
fication. These  persons  have  especially  made  themselves  unfit  to  bear 
abstinence. 

"What  is  important  for  the  understanding  of  the  anxiety  neurosis 
is  the  fact  that  any  noteworthy  development  of  the  same  occurs  only 
in  men  who  remain  potent,  and  in  non-anesthetic  women.  In  neuras- 
thenics, who  on  account  of  masturbation  have  markedly  injured  their 
potency,  anxiety  neurosis  as  a  result  of  abstinence  occurs  but  rarely 
and  limits  itself  usually  to  hypochondria  and  light  chronic  dizziness. 
The  majority  of  women  are  really  to  be  considered  as  'potent;'  a 
real  impotent,  that  is,  a  real  anesthetic  woman,  is  also  inaccessible  to 
anxiety  neurosis,  and  bears  strikingly  well  the  injuries  cited. 

"How  far  we  are  perhaps  justified  in  assuming  constant  relations 
between  individual  etiological  moments  and  individual  symptoms  from 
the  complex  of  anxiety  neurosis,  I  do  not  care  to  discuss  here. 

"  (/)  The  last  of  the  etiological  determinants  to  be  mentioned  seems, 
in  the  first  place,  really  not  to  be  of  a  sexual  nature.  Anxiety  neurosis 
originates  in  both  sexes  through  overwork,  exhaustive  exertion,  as, 
for  instance,  after  sleepless  nights,  nursing  the  sick,  and  even  after 
serious  illnesses." 

Neurasthenia. — The  term  neurasthenia,  since  it  first  came  into  use 
only  a  little  over  a  generation  ago,  has  been  applied  to  almost  every 
conceivable  condition.  Almost  all  illnesses  are  accompanied  by  a  certain 
amount  of  easy  fatigability,  emotional  instability,  and  a  general  out- 
of-sorts  feeling.  All  combinations  of  this  kind  which  cannot  be  specific- 
ally diagnosed  and  placed  under  some  well-known  caption  are  easily 
dropped  into  the  miscellaneous  group  of  neurasthenia.  Not  only  have 
all  sorts  of  conditions,  therefore,  been  included  under  this  term,  but 
the  most  varied  symptoms  have  been  thereby  designated  as  neuras- 
thenic. Not  only  have  many  physical  conditions  been  included,  such 
as  general  arteriosclerosis,  but  it  is  not  uncommon  for  some  of  the  more 


NEURASTHENIA  621 

serious  psychoses,  especially  in  their  milder  manifestations,  as  the 
cyclothymias,  to  be  diagnosed  as  neurasthenia.  It  is  highly  desir- 
able, therefore,  to  limit  the  application  of  the  term  to  a  definite 
condition. 

It  is  better  to  consider  neurasthenia  as  the  expression  of  a  very 
marked  auto-erotic  fixation,  as  a  return  to  that  infantile  period  of 
development  in  which  the  child  takes  a  preponderating  interest  in 
its  own  body.  Masturbation  is  quite  liable  to  be  indulged  in  as  a 
means  of  auto-erotic  satisfaction  but  the  physical  act  of  masturbation 
is  perhaps  relatively  unimportant  as  compared  with  the  crippling 
effects  of  the  auto-erotic  introversion. 

This  condition  is  known  as  a  primary  fatigue  neurosis  and  has  cer- 
tain quite  characteristic  and  constant  symptoms  which  are  in  the 
main  a  feeling  of  pressure  on  the  top  of  the  head,  more  or  less  insomnia, 
spinal  irritation,  with  perhaps  pain  in  the  back,  certain  paresthesias, 
easy  fatigability,  emotional  irritability,  and  some  depression. 

This  condition,  despite  outward  evidence  to  the  contrary,  has  been 
traced  in  most  instances  where  careful  analysis  of  the  symptoms  has 
been  made,  to  a  specific  sexual  etiology,  namely  to  excessive  mastur- 
bation or  frequent  pollutions.  In  contrast  to  the  etiology  of  the  anxiety 
neurosis,  which,  as  has  been  said,  is  dependent  upon  an  inadequate 
utilization  and  incomplete  discharge  of  the  energy  of  the  sexual  act 
in  the  psychic  sphere,  in  neurasthenia  the  specific  etiology  is  dependent 
upon  an  inadequate  discharge  in  the  physical  sphere. 

One  has  to  think  in  addition  to  the  specific  etiology  of  the  fact  that 
in  most  instances  where  masturbation  is  practised  into  adulthood  there 
is  a  serious  moral  conflict.  The  individual  feels  ashamed,  chagrined, 
humiliated  by  having  yielded  to  the  physical  demand.  This,  of  course, 
adds  to  the  difficulty  by  increasing  the  amount  of  energy  dissipated. 
In  addition  to  this  the  moral  conflict  is  usually  very  greatly  enhanced 
either  by  being  told  or  reading  of  the  awful  results  of  this  habit.  This 
is  especially  so  when  these  results  are  told  to  the  child  in  order  to 
frighten  him  into  desisting  and  are  accompanied  by  threats  of  cutting 
off  the  organ  and  the  like. 

It  must  not  be  lost  sight  of  in  dealing  with  this  class  of  patients  that 
a  moderate  amount  of  masturbation  during  infancy,  about  the  third 
or  fourth  year,  at  the  end  of  what  Freud  calls  the  first  latency  period, 
is  normal  and  probably  has  as  its  function  the  focalization  of  the 
sexual  erethism  upon  the  sex  organs.  It  will  be  remembered  that 
before  this  time  the  various  erogenous  zones  of  the  body  such  as  the 
sex  organs,  the  lips,  the  anus,  are  of  practically  equal  significance.  For 
the  function  of  reproduction  the  sex  organs  must  emerge  with  a  pre- 
ponderant erethism,  otherwise  some  one  of  the  perversions  will  take 
the  place  of  normal  sexuality.  It  seems,  therefore,  the  function  of 
masturbation  to  help  produce  this  result. 

When  masturbation,  however,  is  indulged  in  about  the  period  of 
puberty  and  later  on  into  adult  life  it  has  certain  dangers  in  addition 


622  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

to  those  which  are  more  nearly  at  the  physiological  level  and  which 
are  productive  of  neurasthenia.  The  individual  in  his  psychosexual 
development  passes  through  an  auto-erotic  period  when  he  finds  his 
sexual  interests  in  himself,  then  through  a  period  in  which  his  sexual 
interests  are  transferred  to  the  immediate  members  of  the  family,  the 
period  of  narcissism  in  which  at  first  he  is  most  interested  in  those 
members  of  the  family  most  like  himself,  namely  of  the  same  sex. 
Passing  through  this  homosexual  and  narcissistic  period  he  finally 
reaches,  after  having  passed  the  period  of  puberty,  to  the  possibility 
of  giving  his  love  out  to  someone  else,  not  only  besides  himself,  but 
someone  removed  from  the  family  circle  and  someone  of  the  opposite 
sex.  He  becomes  normally  heterosexual  and  attains  the  period  of 
object  love.  Now  one  of  the  serious  dangers  of  masturbation  is  the 
danger  it  has  of  fixing  the  individual  at  some  intermediate  point  in 
his  psychosexual  development.  The  principal  danger  is,  of  course, 
fixation  at  the  infantile  auto-erotic  period,  which  is  naturally  the  par- 
ticular quality  of  sexuality  that  masturbation  ministers  to.  This 
fixation  not  only  prevents  the  proper  psychosexual  development,  but 
drags  the  whole  personality  back  upon  itself  and  prevents  that  open, 
free,  and  outward  manifestation  which  is  essential  to  success  in  life,  to 
a  finding  of  one's  place  in  the  world.  These  people  are  too  thoroughly 
occupied  with  themselves  to  be  able  to  deal  with  the  outside  world  of 
reality  with  any  degree  of  efficiency. 

In  the  act  of  masturbation  the  individual  is  both  the  subject  and 
the  object.  He  has  to  supply  the  energies  from  both  sources,  not  only 
the  energies  from  within,  but  all  of  the  energies  and  stimuli  which 
normally  would  come  from  without  from  the  person  of  another.  During 
his  indulgences  he  frequently  develops  all  manner  of  phantasies  and 
these  phantasies  often  throw  a  considerable  light  upon  the  nature  of 
his  psychosexual  development,  quite  similarly  as  do  the  dreams  in 
those  patients  who  suffer  from  frequent  pollutions. 

It  will  be  seen,  therefore,  that  in  neurasthenia  there  is  a  condition 
that  is  by  no  means  simple  and  that  requires  careful  analytical  study 
in  order  to  be  able  to  deal  with  it  intelligently. 

A  final  word  as  to  the  causes  of  neurasthenia:  The  alleged  causes 
of  this  disorder  have  been  as  multiform  as  the  conditions  which  have 
been  ranged  under  it.  There  are  a  large  group  of  cases  which  are  sup- 
posed to  be  dependent  upon  injuries,  traumatic  neurasthenia,  and 
another  large  group  that  is  supposed  to  be  dependent  upon  overwork. 
Although  it  is  not  quite  possible  to  speak  dogmatically  with  regard  to 
the  traumatic  group  at  this  time,  still  from  analogy,  as  the  result  of 
cases  studied  in  the  group  supposed  to  be  dependent  upon  overwork,  it 
will  be  seen  that  the  same  reasoning  applies  to  both  and  it  is  again  the 
same  reasoning  that  may  apply  to  anxiety  neurosis,  or  in  fact  to  any 
of  the  conditions  described  in  this  chapter,  but  more  particularly 
perhaps  to  the  actual  neuroses.  The  traumatism  or  the  overwork,  as 
the  case  may  be,  or  any  other  apparent  assigned  cause  can  be  said  not 


MIXED  NEUROSES  623 

to  be  the  true  cause  of  the  neurosis,  but  only  its  occasion.  The  trau- 
matism or  the  overwork  could  not  produce  the  neurosis  in  the  absence 
of  the  specific  etiology.  It  is  quite  understandable  that  a  given  indi- 
vidual may  stand  a  series  of  sexual  traumatisms  over  a  considerable 
period  of  time,  but  be  strong  enough  to  resist  the  development  of  a 
neurosis.  On  the  occasion,  however,  of  having  his  resistance  reduced 
as  the  result  of  an  injury,  or  as  the  result  of  long-continued  overwork 
the  neurosis  crops  out.  This  is  the  explanation  for  many  of  these  con- 
ditions, and  it  is  the  reason  why  a  banal  cause  may  develop  a  result 
that  is  out  of  all  proportion  in  both  quantity  and  quality. 

Mixed  Neuroses. — Hysteria,  the  compulsion  neurosis,  anxiety 
neurosis,  and  neurasthenia  have  been  described.  If  the  etiology  and 
mechanisms  of  these  four  conditions  be  considered  it  will  be  seen  that 
they  do  not  of  necessity  mutually  exclude  one  another  and  as  a  matter 
of  fact  not  infrequently  certain  admixtures  are  found  in  clinical  expe- 
rience. Anxiety  hysteria,  for  example,  has  taken  a  rather  definite  place 
among  these  conditions,  while  as  will  be  readily  seen  from  the  nature 
of  the  etiological  moments  neurasthenia  and  anxiety  neurosis  are  not 
infrequently  found  associated  in  various  proportions,  while,  of  course, 
it  follows  that  the  etiological  moments  of  the  actual  neuroses  are 
not  excluded  from  operating  in  the  same  patient  who  may  have  a 
psych  oneurosis.  Such  combinations  are,  therefore,  of  not  uncommon 
occurrence. 

Aside  from  the  possibility  of  the  mixture  of  the  neuroses  it  should 
also  not  be  lost  sight  of  that  the  etiological  moments  of  the  neuroses 
may  also  operate  in  persons  who  are  suffering  from  the  more  severe 
psychoses,  as  for  example,  manic-depressive  psychosis  and  dementia 
precox.  We  quite  commonly  find  hysterical  symptoms  in  the  precox, 
while  neurasthenic  and  anxiety  states  are  not  infrequent  in  the  depres- 
sions of  manic-depressive  psychosis.  Other  combinations,  of  course, 
might  be  mentioned.  The  important  thing  to  bear  in  mind,  however,  is 
the  nature  of  the  etiological  moment  and  the  mechanism  of  the  con- 
dition, and  then  these  will  be  recognized  when  the  individual  patient 
is  under  analytic  observation. 

Finally,  the  purely  psychic  element  is  found  more  and  more  in 
association  with  the  actual  neuroses.  This  was  intimated  in  the  de- 
scription of  the  etiology  and  mechanisms  of  neurasthenia  and  it  has 
also  been  spoken  of  in  the  description  of  the  anxiety  neurosis.  More 
will  be  said  on  this  point  under  the  head  of  Treatment. 

Treatment  of  the  Neuroses. — In  the  treatment  of  the  actual  neuroses 
the  main  thing,  as  indicated  by  the  description  which  has  been  given, 
is  to  correct  the  sexual  life  of  the  patient.  In  dealing  with  neuras- 
thenia the  habit  of  masturbation,  if  it  be  present,  must,  of  course, 
be  stopped  before  anything  definite  can  be  accomplished,  while,  of 
course,  with  both  neuroses  matters  of  coitus  interruptus,  ejaculatio 
precox,  abstinence,  etc.,  must  be  carefully  inquired  into  and  the  sexual 
life  modified  as  indicated  so  that  the  evil  effects  which  result  from  them 


624  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

may  be  remedied.  These  are  the  simple  things  to  do,  and  in  many 
cases  will  produce  marked  betterment,  if  not  apparent  recovery. 

Rest  cures,  hydrotherapy,  massage,  electricity,  exercise,  and  all 
such  therapeutic  agents  have  their  place  in  the  treatment  of  the 
neuroses,  particularly  the  actual  neuroses,  but  their  place  is  a  second- 
ary one.  It  has  already  been  indicated  that  the  precipitating  factor 
in  the  outbreak  of  a  neurosis  may,  for  example,  be  overwork,  but  that 
the  neurosis  would  not  eventuate  in  the  absence  of  the  specific  etiology. 
The  explanation  of  this  occurrence  was  that  the  patient  was  strong 
enough  to  stand  up  under  the  results  of  sexual  traumata  until  his  gen- 
eral resistance  was  reduced  by  overwork  and  then  the  neurosis  appeared. 
It  will  be  seen,  therefore,  that  the  usual  methods  of  treatment  very 
frequently  bring  about  a  cure,  but  not  in  the  way  in  which  they  are 
supposed  to.  By  changing  the  patient's  sexual  habits,  removing  him 
from  his  surroundings,  sending  him  away  to  a  sanitarium,  oftentimes 
the  sexual  situation  is  more  or  less  well  solved  temporarily.  Now,  if 
during  this  period  of  residence  in  a  sanitarium  he  is  on  careful  diet, 
given  regular  exercise  with  baths  and  massage,  it  is  perfectly  under- 
standable that  his  general  resistance  will  be  increased  so  that  he 
may  overcome  the  effects  of  the  sexual  traumata.  In  this  way  he  may 
get  well  without  any  direct  attack  upon  the  factors  of  the  specific 
etiology.  This  is  the  principle  which  is  oftentimes  seen  in  operation  in 
the  improvement  and  alleged  recoveries  of  neurotics  as  a  result  of 
the  rest  cure. 

Inasmuch,  however,  as  even  the  actual  neuroses  usually  present 
some  admixture  of  purely  mental  symptoms,  either  primary  or  second- 
ary in  origin,  it  may  be  necessary  ultimately  and  before  satisfactory 
results  can  be  obtained  to  deal  with  these  mental  symptoms.  If  so, 
they  must  be  dealt  with  in  precisely  the  same  way  as  the  mental 
symptoms  of  the  psychoneuroses,  namely  by  psychoanalysis. 

The  main  principle  involved  in  psychoanalysis  may  be  said  to  be 
an  analysis  of  the  patient's  mental  condition  sufficiently  complete  to 
thoroughly  understand  the  symptomatic  manifestations  of  his  malady. 
Before  psychoanalytic  methods  were  employed  no  explanation  was 
sought  for  mental  symptoms  and  apparently  it  rarely  entered  anyone's 
mind  that  they  had  any.  The  patient  who  had  a  phobia  or  a  tic  was 
simply  looked  upon  as  being  nervous,  perhaps  having  had  some  fright 
or  bad  impression  earlier  in  life,  and  was  usually  treated  by  tonics  or 
rest  or  travel  or  some  other  such  means  that  was  not  addressed  to 
the  solution  of  the  problem  in  any  way.  From  what  has  already  been 
said  about  the  psychic  development  of  the  individual  it  will  be  readily 
appreciated  that  no  mental  fact  can  fail  to  have  a  thoroughly  logical 
and  understandable  reason  for  its  existence,  and  it  is  the  object  of 
psychoanalysis  to  find  this  out. 

Briefly,  the  technique  of  psychoanalysis  is  about  as  follows,  being 
of  course,  modified  in  detail  to  some  extent  by  the  exigencies  of  the 
occasion  and  as  the  result  of  the  special  predilections  of  the  physician 


MIXED  NEUROSES  625 

practising  it.  When  the  patient  calls  upon  the  physician  the  physician 
should  let  the  patient,  as  far  as  possible  without  interrupting  him  by 
questions  or  otherwise,  detail  to  him  his  difficulties.  This  may  take 
only  a  few  minutes,  or  may  be  quite  a  lengthy  recital,  but  it  is  usually 
worth  while  to  listen  carefully  to  the  whole  thing,  perhaps  occasionally 
by  a  suggestion,  keeping  the  patient  to  the  point  if  he  tends  to  be  too 
circumstantial.  This  original  statement  contains  a  description  of  the 
things  from  which  the  patient  is  suffering,  and  if  careful  attention  is 
paid  to  it  one  may  get  many  hints  as  to  how  to  pursue  the  further 
inquiry.  As  a  result  of  this  conversation  and  perhaps  another,  if  it  is 
necessary,  the  physician  makes  up  his  mind  whether  the  patient's 
illness  is  a  suitable  one  for  psychoanalytic  treatment,  and  if  it  is  he  so 
states  and  then  makes  arrangements  for  regular  consultations,  prefer- 
ably not  less  than  three  times  per  week,  of  an  hour's  duration  each,  and 
all  other  preliminaries  such  as  the  fee,  etc.,  are  arranged  too.  Should 
it  appear  that  the  patient  has  any  symptoms  of  physical  illness  he 
should  be  sent  to  a  competent  physician  skilled  in  the  particular  trouble 
that  appears  to  be  present,  for  the  psychoanalyst  should  under  no 
circumstances  undertake  to  treat  the  physical  condition.  The  reasons 
for  this  will  appear  later.  It  is  preferable,  too,  that  the  physical  con- 
dition be  attended  to  fully  before  the  psychoanalytic  treatment  be 
taken  up.  In  other  words,  it  is  undesirable  to  endeavor  to  do  psycho- 
analysis while  the  patient  is  under  the  care  of  another  physician. 

Having  arranged  all  the  preliminaries,  the  patient  calls  at  the 
appointed  hour  and  the  psychoanalytic  conversation  proceeds  about 
as  follows:  Bearing  in  mind  the  ultimate  goal,  the  psychological 
explanation  of  the  patient's  symptoms,  the  patient  may  be  approached 
by  beginning  the  discussion  of  one  of  the  symptoms,  either  simply 
asking  about  it  or  else  pursuing  some  line  of  inquiry  that  was  suggested 
in  the  original  conversation.  On  the  other  hand,  the  method  may  be 
pursued  of  endeavoring  to  first  get  a  clear  understanding  of  the  whole 
life  of  the  patient,  beginning  from  the  earliest  recollections  and  trac- 
ing the  development  to  the  present.  It  really  makes  very  little  differ- 
ence how  one  starts,  because  in  a  very  short  time  there  will  be  all 
manner  of  suggestions  to  develop  inquiries  along  various  lines,  and 
these  will  have  to  be  followed  out  for  a  complete  untangling  of  the 
situation. 

During  the  course  of  the  psychoanalytic  conversations  one  will  get 
very  shortly  to  a  point  from  which  progress  seems  to  be  impossible, 
for  it  does  not  take  long  to  exhaust  the  consciousness  of  the  patient. 
One  then  has  to  penetrate  the  foreconscious,  which  is  relatively  easy, 
and  the  unconscious,  which  is  relatively  difficult.  The  method  of 
procedure  here  is  the  method  by  free  association.  Perhaps  a  point  has 
been  attained  in  the  conversation,  a  situation  has  been  unfolded,  which 
has  no  apparent  explanation.  The  patient  cannot  give  any  reasons  which 
adequately  account  for  it.  Under  these  circumstances  the  patient  is 
asked  to,  so  to  speak,  take  the  situation  as  a  starting-point,  and  then 
40 


626  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

relaxing  into  a  condition  of  perfect  passivity  observe  the  thoughts  that 
come  to  his  mind  and  recount  them  as  fast  as  they  appear.  In  other 
words,  he  is  asked  to  place  himself  in  a  mental  state  favorable  to  phan- 
tasy formation,  he  is  asked  to  relinquish  his  grasp  upon  his  mental 
life,  to  permit  his  ideas  to  flow  untrammelled  and  unselected  by  his 
volition  and  critique.  He  is  asked  as  it  were  to  become  the  observer 
of  his  own  ideas,  to  sit  as  if  he  were  sitting  in  the  window  of  a  moving 
train,  recounting  aloud  the  objects  as  they  passed  by.  So  he  is  asked  to 
observe  his  ideas  and  to  tell  them  as  they  come.  This  sounds  like  rather 
a  simple  procedure,  but  it  is  a  very  difficult  one  for  the  patient  to 
learn,  and  in  fact  when  the  patient  can  do  it  and  do  it  easily  he  is 
approaching  the  end  of  his  treatment. 

Experience  shows  that  when  patients  are  instructed  in  this  way 
they  take  the  situation  as  directed  as  a  starting-point,  and  with  the 
intention  of  telling  the  ideas  that  come  to  their  mind  they  will  relax 
into  a  condition  of  passivity.  Perhaps  then  for  a  considerable  time 
they  do  not  speak,  and  if  they  are  asked  why  they  do  not  tell  what 
comes  to  their  mind  they  will  say  that  nothing  comes.  This  is,  of 
course,  the  interference  of  the  repressed  complexes;  it  is  the  resistance 
which  they  have  to  coming  into  consciousness  which  is  manifesting 
itself  in  this  way.  The  whole  thing,  then,  has  to  be  gone  over  with  the 
patient.  It  has  to  be  explained  to  them  that  their  mind  cannot  be  an 
absolute  blank,  and  they  have  to  be  warned  especially  not  to  exercise 
choice  as  to  what  ideas  they  shall  tell  and  what  they  shall  not  tell, 
that  it  makes  no  difference  how  absurd  or  inconsequential  the  idea  is 
that  comes  to  their  mind  they  must  tell  it;  even  if  the  idea  is  extremely 
disagreeable  they  must  tell  it,  for  no  matter  how  little  connection  it 
may  appear  to  have  with  their  trouble  if  the  starting-point  has  been 
from  some  problem  in  the  case  these  ideas  that  come  must  have  some 
connection  with  that  problem.  It  must  be  explained  to  them  that,  of 
course,  they  cannot  see  the  connection,  but  that  they  must  tell  the 
ideas  so  that  the  physician  may  have  them  and  that  he  will  be 
able  to  see  what  bearing  they  have  in  the  situation.  Of  course  he  may 
not  be  able  to  see  at  once,  but  it  is  so  much  material  which,  if  it  does 
not  come  in  for  utilization  today,  can  be  used  perhaps  tomorrow,  or 
the  day  after. 

This  is  the  process  of  free  association,  one  which  is  very  difficult 
for  the  patient  to  learn  and  one  which  requires  much  skill  and  no 
little  art  on  the  part  of  the  physician.  The  physician  must  be  ever 
on  the  alert.  It  requires  the  most  intense  application  to  the  question 
in  hand,  for  everything  must  be  watched  with  the  utmost  care.  Every 
little  detail  must  be  observed  as  containing  perhaps  a  hidden  meaning 
behind  it.  The  hesitations,  the  stammerings,  the  mistakes,  the  slips 
of  the  tongue,  all  have  their  significance.  For  example,  in  talking  to  a 
young  man,  who  told  about  his  previous  illness,  he  said  that  the  phy- 
sician had  prescribed  four  quarter-grain  tablets  of  "quinin"  for  him. 
He  had  no  sooner  mentioned  the  name  of  the  drug  than  he  immediately 


MIXED  NEUROSES  627 

corrected  himself  and  said  "calomel."  An  inquiry  into  the  meaning 
of  quinin  to  him  brought  out  a  most  important  event  in  his  life,  an 
event  undoubtedly  of  significance  in  his  neurosis.  The  repressed 
complex  was  struggling,  as  it  always  is,  for  expression.  A  favorable 
opportunity  presented  itself.  Calomel  and  quinin  are  easily  mixed 
in  the  pronunciation,  and  the  repression  slipped  its  moorings  for  a 
moment  and  found  expression.  It  remained  for  the  observer  to  be 
sufficiently  keen  to  see  the  possibility  of  meaning  in  such  a  mistake 
and  find  out  that  meaning. 

The  most  important  single  aid  in  determining  the  content  of  the 
unconscious  is  the  dream.  The  split-off  complexes  are,  in  accordance 
with  the  theory  set  forth,  actively  repressed  by  the  individual,  they 
are  not  permitted  to  come  to  expression  if  he  can  help  it.  They  there- 
fore can  only  express  themselves  in  symbolic  form,  in  which  form  they 
are  disguised  not  only  to  others,  but  to  the  patient  himself.  In  this 
disguised  form  they  are  not  recognized  for  what  they  are,  and  there- 
fore the  painfulness  of  their  recall  to  consciousness  is  avoided.  A 
previous  moral  delinquency  may  thus  come  to  the  surface  under  a 
complete  disguise  without  causing  any  particular  distress,  whereas 
it  would  be  quite  unbearable  if  it  came  forth  in  its  true  colors.  It  can 
be  seen  from  this  why  it  is  that  obstacles  are  so  quickly  reached  in 
the  psychoanalytic  procedure.  The  dream  is  the  best  and  most  useful 
avenue  for  overcoming  this  type  of  obstacle.  Here  the  repressed 
complexes  come  upon  the  stage  in  all  their  paraphernalia  of  symbolic 
disguise  and  find  an  opportunity  for  expression.  The  patient,  not 
understanding  what  the  dream  means,  will  pretty  generally  recount 
it  in  all  its  details,  a  thing  which  he  would  absolutely  refuse  to  do,  in 
many  instances  at  least,  if  he  had  the  slightest  suspicion  of  what  it 
could  mean.  The  physician  is  therefore,  so  to  speak,  in  a  position 
to  come  up  on  the  blind  side  of  the  patient,  to  see  the  play  of  his 
unconscious  phantasies,  to  be  let  behind  the  scenes,  as  it  were.  Now  if 
he  can  penetrate  these  disguises  then  he  not  only  is  capable  of  deter- 
mining the  meaning  of  the  dream,  but  also  he  learns  in  this  way  the 
nature  of  the  repressed  complexes,  and  is  therefore  in  a  position  to 
begin  to  read  meaning  into  the  symptoms  of  the  neurosis.  The  whole 
matter  of  dream  interpretation  constitutes  a  special  chapter  in  psy- 
chology and  is  hardly  discussable  in  a  text-book  of  this  sort  with  its 
necessary  limitations. 

In  addition  to  the  dream  analyses  one  should  also  inquire  into  the 
phantasy  formations  of  the  patient,  phantasies  which  are  formed 
in  the  day  time.  They  are  interpreted  on  the  same  principles  as  the 
dream. 

The  events  uncovered  by  the  method  of  free-association  must  be 
dealt  with  as  facts.  It  makes  little  difl^erence  whether  they  even  did 
happen  in  reality  or  not  they  are  nevertheless  psychoJocjical  facts; 
they  represent  the  way  the  patient  thinks  and  so  have  just  as  much 
value  as  if  they  represented  real  occurrences. 


628  THE  PSYCHONEIJ ROSES  AND  ACTUAL  NEUROSES 

It  will  be  seen,  therefore,  that  psychoanalysis  is  a  lengthy,  pains- 
taking, detailed  dissection  of  the  mind  of  the  patient  sufficient  for 
the  explanation  of  the  symptoms.  This  dissection  starts  at  the  surface 
and  may  go  to  practically  any  depth.  Bearing  in  mind  the  principles 
already  elucidated  it  will  be  seen  why  it  is  possible  to  effect  an  appar- 
ent cure  at  various  levels;  why  sometimes  a  single  conversation  may 
apparently  produce  the  miracle  of  a  cure,  while  in  other  patients  a  half- 
dozen  will  produce  the  same  effect,  and  in  still  others  months  of  care- 
ful work  are  required.  The  individual  has  been  thrown  out  of  adjust- 
ment by  causes  which,  in  their  last  analysis,  have  been  operative  the 
greater  part  of  his  life.  Up  to  a  certain  point,  however,  up  to  a  certain 
difficulty,  he  has  been  able  to  get  along.  Now,  when  this  difficulty 
comes  he  breaks  and  the  neurosis  makes  its  appearance.  -If  he  can 
be  patched  up,  so  to  speak,  rehabilitated  back  to  the  point  where 
the  break  occurred,  an  apparent  cure  results.  For  these  apparent 
cures  it  will  be  seen  that  it  is  only  necessary  to  carry  the  patients  back 
to  a  point  at  which  they  are  capable  of  making  adjustment.  This 
is  what  very  often  happens,  especially  in  methods  of  treatment  other 
than  the  psychoanalytic.  For  a  real  cure  of  the  patient,  however, 
something  very  much  more  radical  than  this  is  required,  that  the 
analyst  has  to  proceed  to  ever  and  ever  deeper  levels  until  he  has 
sought  out  and  found  the  final  stronghold  of  the  neurosis.  Anything 
short  of  this  can  only  serve  to  effect  a  compromise. 

This  seems  to  be  the  most  advantageous  point  to  answer  the  ques- 
tion which  is  constantly  being  asked  and  which  it  seems  impossible 
to  make  many  people  understand,  namely,  the  question  of  how  the 
analysis,  the  unraveling  of  the  symptoms,  produces  a  cure.  In  order 
to  understand  this  it  is  necessary  to  recur  to  the  statement  that  the 
illness  is  due  to  a  conflict  and  that  one  element  of  the  conflict  is 
unconscious  to  the  patient;  he  does  not  know  what  it  is  that  he  is 
fighting.  He  therefore  cannot  deal  with  it  frankly,  openly,  intelli- 
gently. One  is  reminded  of  the  story  of  a  certain  king  who  propounded 
the  question  to  the  wise  men  as  to  why  a  bowl  of  water  was  not 
increased  in  weight  when  a  live  fish  was  put  into  it,  while  it  was 
increased  in  weight  when  a  dead  fish  was  put  into  it.  This  created  a 
tremendous  disturbance.  All  sorts  of  arguments  and  reasons  were 
propounded,  heated  discussions  arose,  and  the  wise  men  were  quite 
generally  out  of  tune  with  one  another.  Finally,  it  occurred  to  some 
one  to  try  the  experiment  and  see  what  the  facts  were.  As  soon  as 
the  experiment  was  tried  and  the  facts  were  determined  there  was  no 
longer  any  cause  for  argument.  The  conflict  subsided.  This  is  quite 
comparable  to  the  position  in  which  the  patient  finds  himself,  fighting 
something  which  is  unconscious  and  about  which  he  knows  nothing. 
The  facts  in  the  case  in  regard  to  the  fish  in  the  bowl  of  water  were 
unknown,  and  as  long  as  they  were  unknown  nothing  but  chaos 
reigned  among  the  wise  men.  As  soon  as  the  facts  were  brought  to 
light,  however,  by  proper  experimentation,  then  there  was  no  longer 


MIXED  NEUROSES  629 

anything  to  fight  about.  In  addition  to  these  reasons  for  the  sub- 
sidence of  the  conflict  there  are  others  that  are  of  more  or  less  impor- 
tance in  different  cases;  among  them  is  a  large  element  of  reeducation 
to  which  the  patient  is  subjected  throughout  the  period  of  psycho- 
analysis. The  cause  of  his  neurosis  implies  that  he  is  somewhat 
infantile,  somewhat  undeveloped.  The  physician,  who  should  be  a 
man  of  wide  learning,  whose  business  it  is  to  deal  with  the  problem  of 
right  living,  cannot  help  but  infuse  into  the  patient  in  the  many 
hours  of  conversation  a  philosophy  of  life  which  is  helpful,  and  this 
undoubtedly  happens  as  one  of  the  most  important  elements  in  the 
reeducation,  development  and  rehabilitation  of  the  patient. 

While  the  simple  uncovering  of  the  meaning  of  a  symptom  is  often 
enough  to  make  it  disappear,  for  the  real  rehabilitation  of  the  patient 
the  whole  meaning  of  infantile  ways  of  reacting  must  be  finally  under- 
stood and  the  patient  must  be  willing  to  forego  this  means  of  obtaining 
pleasure  in  order  to  advance  to  a  higher  level  of  adjustment,  to  solve 
the  difficulties  by  attaining  a  higher  level  which  means  at  once 
renunciation  and  fulfilment. 

And  finally,  about  the  matter  of  transference.  The  neurotics  and 
the  psychoneurotics  and  the  vast  majority  of  persons  who  require 
psychotherapeutic  treatment,  are  introverted,  that  is,  their  interests 
are  turned  within,  upon  themselves,  and  they  cannot  be  made  over 
into  efficient  people  capable  of  dealing  with  the  outside  world  of 
reality  until  their  interests  can  be  made  to  flow  outside  of  themselves, 
until  they  can  become  interested  in  persons  and  things  and  events. 
In  the  course  of  psychoanalysis,  if  it  is  to  proceed  successfully,  one  of 
the  earliest  things  that  happens  is  that  the  interest  of  the  patient 
begins  to  flow  upon  the  physician.  It  is  transferred  to  him.  This  is 
a  matter  of  utmost  importance.  It  is  a  matter  which  should  be 
watched  with  the  greatest  care,  for  it  is  the  barometer  of  the  relation- 
ship between  physician  and  patient.  It  is  the  factor  in  the  personal 
equation  which  plays  such  a  great  part  and  which  was  supposed  in 
the  old  days  to  play  practically  the  only  part.  As  soon  as  transfer 
begins  to  take  place  then  the  patient  will  begin  to  bring  dreams  for 
analysis,  and  in  other  ways  to  show,  so  to  speak,  every  desire  to  please 
the  physician  by  doing  as  he  wishes,  and  before  long  one  will  generally 
find  that  the  dreams  are  occupied  with  the  idea  of  the  physician,  they 
are  transfer  dreams.  Now  in  these  dreams  one  may  find  exactly  how 
the  physician  is  held  in  the  mind  of  the  patient.  In  symbolic  form 
the  transfer  dream  may  indicate  that  the  physician  is  held  in  high 
regard  and  that  he  is  respected,  and  this  is  of  course  as  it  should  be. 
On  the  other  hand,  he  may  have  failed  to  deal  with  a  certain  situation 
adequately,  and  the  dream  will  show  that  the  patient  is  disappointed 
or  that  perhaps  some  idea  that  the  physician  suggested  the  patient 
thinks  is  ridiculous  and  silly,  or  the  physician  may  make  the  mistake 
of  talking  over  the  head  of  the  patient  so  that  the  patient  cannot 
understand,  and  the  dream  will  say  what  the  patient  could  not  say 


630         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

himself,  that  it  is  all  too  deep  for  him,  that  he  cannot  follow,  and  that 
the  physician  is  altogether  beyond  him  in  the  whole  matter,  and  it 
all  seems  quite  hopeless.  The  transfer  dream  therefore  becomes  a 
very  important  matter  and  requires  the  physician  constantly  to  look 
within  and  to  exercise  his  self-critique,  for  he  must  always  realize 
that  if  he  fails  at  a  certain  point  the  trouble  is  not  with  the  patient, 
but  with  himself,  and  sometimes  the  dream  will  indicate  what  the 
trouble  is.  The  meaning  of  the  transfer  is  of  course  that  the  patient 
cannot  unburden  his  very  soul,  cannot  stand  unclothed  in  all  the 
nakedness  of  his  real  self,  cannot,  in  other  words,  completely  confess 
himself  to  an  indifferent  person.  There  must  be  something  in  the 
physician  which  commands  the  patient's  affection,  respect  and  con- 
fidence, and  therefore  the  psychoanalytic  work,  while  it  makes  great 
demands  upon  the  patient  also  makes  great  demands  upon  the 
physician. 

Sometimes  the  transfer  is  of  such  a  character  and  sufficiently  intense 
of  itself  to  interfere  with  the  progress  of  the  analysis.  Under  these 
circumstances  it  must  be  discussed  with  the  patient,  sufficiently 
discussed  to  do  away  with  the  resistances  it  has  raised,  and  finally  at 
the  completion  of  the  analysis  the  whole  matter  of  the  transfer  must 
be  freely  gone  into,  and  analyzed,  so  that  there  is  no  misunderstanding 
as  to  what  its  real  meaning  might  be.  The  patient  is  to  be  placed 
in  full  and  complete  possession  of  all  of  the  facts,  which  means  a  full 
and  complete  possession  of  himself.  Nothing  must  be  hidden  from 
him,  the  mirror  must  be  held  up  so  that  he  can  see  himself  in  it  in  every 
detail. 

It  is  because  of  the  necessity  of  transfer  to  successful  psychoanalytic 
handling  of  a  case  that  it  is  undesirable  to  have  another  physician 
treating  the  patient  at  the  same  time.  The  other  physician  might 
acquire  the  transfer,  and  this  would  prevent  the  psychoanalyst  from 
accomplishing  anything.  This  is  especially  apt  to  be  the  case  where 
the  other  physician  has  to  do  with  the  physical  condition  of  the 
patient  and  has  to  come  into  personal  contact  with  him  in  making 
examinations  and  the  like. 

As  already  said,  the  transfer  is  the  result  of  the  beginning  flowing 
outward  of  the  patient's  interests  into  the  world  of  reality.  The 
physician  naturally  is  the  one  toward  whom  this  interest  first  flows. 
He  therefore,  so  to  speak,  puts  himself  in  a  position  to  be  utilized 
by  the  patient;  he  becomes  a  bridge  by  which  the  patient  is  able  to 
get  back  into  the  world.  At  the  completion  of  the  treatment,  when 
the  transfer  has  been  fully  analyzed  and  the  patient  understands 
what  it  means,  then  the  physician  by  so  doing  steps  aside.  Having 
served  the  purpose  of  a  bridge,  having  gotten  the  patient  back  into 
reality,  he  steps  aside  and  leaves  the  patient  there  to  stand  upon  his 
own  feet. 

The  great  obstacle  to  the  psychoanalytic  method  of  treatment  is 
the  general  obstacle  that  the  patient  in  a  certain  real  sense  does 


MIXED  NEUROSES  631 

not  want  to  get  well.  There  are  two  contrary  trends  struggling  for 
supremacy  in  his  psyche.  He  has  two  sets  of  desires  each  trying 
to  gain  fulfilment  and  each  diametrically  opposed  to  the  other.  Now 
the  symptoms  of  his  neurosis  constitute  a  compromise,  and  in  his 
then  state  of  mind  the  only  compromise,  the  only  solution  of  the 
problem  possible  to  him.  He  therefore,  although  he  wants  to  get 
away  from  the  suffering  of  his  neurosis,  still  is  unwilling  to  give  up 
the  symptoms  which  compensate  him,  even  though  that  compensation 
be  inadequate.  We  see  this  illustrated  in  many  waj'^s.  For  example, 
the  patient  insists  upon  leading  the  physician  back  to  infantile  occur- 
rences in  order  to  escape  a  frank  discussion  and  facing  of  present 
problems.  Again,  by  the  free  associations.  The  patient  will  lead  the 
physician  up  all  sorts  of  blind  alleys  for  the  same  purpose.  The 
patient  always  wants  to  avoid  his  task.  It  is  the  function  of  the 
physician  to  hold  him  to  it.  To  this  end  it  is  important  that  the 
physician  should  have  a  wide  knowledge  of  the  meanings  of  symptoms 
and  symbols  and  be  able  fairly  clearly  to  see  at  once  their  significance 
otherwise  he  will  be  led  into  interminable  and  futile  discussions. 
It  is  only  in  the  final  stages  of  the  analysis,  when  the  patient  is  made 
whole,  at  one  with  himself,  that  he  can  understand  why  it  is  that  these 
things  have  come  about  and  how  it  is  that  he  no  longer  needs  his 
illness,  but  can  dispense  with  it.  So  for  a  long  time  the  physician  has 
to  contend  against  an  innate  desire  on  the  patient's  part  to  retain 
the  symptoms  of  his  illness. 

Various  accessory  forms  of  treatment,  such  as  baths,  massage, 
sanitarium  treatment,  travel,  etc.,  have  the  same  place  here  as  with 
the  actual  neuroses  mentioned  before.  They  should  never  be  con- 
sidered primary,  but  only  as  secondary.  If  they  are  utilized  without 
a  thorough  analytic  understanding  of  the  patient  they  are  quite 
as  apt  to  do  harm  as  good;  because  by  no  possibility  can  it  be  forseen 
what  the  results  will  be  unless  the  matter  which  has  to  be  dealt  with 
is  known  beforehand. 

It  must  be  borne  in  mind  that  the  symptoms  of  a  neurosis  or  a 
psychoneurosis  may  cover  and  conceal  a  true  psychosis.  This  will 
appear  in  the  course  of  the  analysis,  but  cannot  always  be  foreseen 
as  the  result  of  the  first  examination.  It  is  a  possibility  that  should 
be  borne  in  mind  so  that  the  physician  may  be  guarded  in  what 
he  says  in  regard  to  the  possibilities  of  treatment  and  the  ultimate 
outcome  of  the  case. 


CHAPTER    XVIL 
MANIC-DEPRESSIVE   PSYCHOSIS. 

The  general  concept  of  the  manic-depressive  psychosis  has  been 
an  extremely  difficult  one  for  some  reason  or  other  for  many  people 
to  adequately  grasp.  From  the  earliest  times  the  marked  cases  of 
melancholia  and  of  maniacal  excitement  have  of  necessity  been 
observed  and  in  many  instances  ably  described,  and  at  one  period  of 
time  the  manias  and  the  melancholias  constituted  by  far  the  larger 
portion  of  the  types  of  mental  disorder.  At  that  time  in  the  history 
of  psychiatry,  when  the  diagnosis  hair-splitting  was  at  its  height,  innu- 
merable varieties  of  mania  and  melancholia  were  described  and  given 
specific  names.  They  were  differentiated  on  the  basis  of  whether 
hallucinations  were  present  or  not,  whether  delusions  were  present  or 
not,  and  upon  like  matters  of  what  seems  now  superficial  observation. 
It  was  observed  also  that  there  were  a  certain  few  cases  in  which 
states  of  excitement  alternated  with  states  of  depression.  These  so- 
called  circular  types  have  been  recognized  for  a  long  time.  It  goes 
without  saying  that  during  this  period  when  psychiatry  was  in  a 
purely  descriptive  stage,  a  stage  from  which  it  has  not  yet  by  any 
means  fully  emerged,  when  the  excitements  and  the  depressions  were 
the  symptoms  in  evidence,  that  excitements  and  depressions  belonging 
to  all  sorts  of  conditions,  dementia  precox,  general  paresis,  arterio- 
sclerosis, toxic  and  infectious  psychoses  were  included  in  the  broad 
concepts  of  mania  and  melancholia  that  were  prevalent  and  that  the 
formulation  of  the  manic-depressive  group  has  been  the  result  of  a 
gradual  weeding  out  from  all  of  these  various  sources  of  the  things 
that  belong  together  and  the  elimination  of  those  which  further 
analysis  showed  were  not  similar. 

The  manic-depressive  concept,  however,  was  not  definitely  for- 
mulated until  Kraepelin,  by  a  study  of  life  histories,  described  the 
condition  as  a  disease  of  affect  fluctuations  which  might  at  one  time 
manifest  itself  by  the  profoundest  depression  and  at  another  time  by 
the  highest  grade  of  excitement.  Even  after  this  formulation  many 
thought  that  the  term  manic-depressive  psychosis  applied  only  to 
the  so-called  cases  of  "circular  insanity"  and  failed  to  appreciate 
that  there  were  inherent  and  fundamental  relations  between  the 
two  extremes  of  affect  disturbance.  It  was  difficult  to  understand 
how  there  could  be  any  relationship  which  bound  together  cases  of 
such  unlike  outward  appearances  and  they  failed  to  see  that  a  patient 
who  manifested  a  single  attack  either  of  depression  or  excitement 


ETIOLOGY  633 

could  be  diagnosed  as  a  manic-depressive  solely  because  the  depres- 
sion or  the  excitement,  as  the  case  might  be,  presented  the  symp- 
tomatology of  the  depression  or  the  excitement  as  found  in  other 
patients  who  presented  a  series  of  attacks  of  both  kinds. 

The  present-day  concept  of  the  manic-depressive  group  considers 
it  as  a  mental  disease  essentially  of  affect  fluctuations  manifesting 
itself  sometimes  by  depression,  sometimes  by  excitement,  sometimes 
by  a  mixture  of  the  two,  and  finally  the  concept  has  grown  to  include 
on  the  one  hand,  a  manic-depressive  character  which  tends  to  react 
on  the  basis  of  a  labile  afi^ectivity,  and  on  the  other  various  atypical 
manifestations  which  present  secondary  symptoms  that  tend  to  mask 
the  fundamental  affective  ones.  And  so  the  concept  emerges  that 
finds  the  root  of  the  psychosis  in  certain  character  traits  that  never 
amount  to  pathological  manifestations,  and  certain  very  mild  affect 
fluctuations,  the  cyclothymias,  and  including  certain  atypical  varieties 
with  secondary  symptoms  that  are  of  greater  practical  significance 
than  the  disturbances  of  affect. 

Here  also,  as  elsewhere,  it  is  seen  that  the  manic-depressive  type  of 
reaction  merges  into  other  types  so  that  we  get  reactions  that  closely 
resemble  the  manic-depressive  in  certain  phases  of  precox,  whereas 
various  of  the  depressions  and  excitements  from  other  causes  also 
closely  parallel  the  manic-depressive  types  in  their  symptomatology. 
With  this  concept  of  reaction  types  in  mind,  these  mergings  into 
adjacent  territories  are  understandable.  With  the  concept  of  a  disease 
entity,  one  which  looks  upon  disease  as  a  definite  something  back  of 
the  symptoms  and  which  produces  them,  it  is  impossible  to  under- 
stand the  meanings  of  these  attenuated  and  border  states. 

Etiology. — In  the  first  place  there  are  certain  hereditary  factors 
to  deal  with  in  this  class  of  cases  as  there  are  in  the  precox  group. 
There  are  certain  families  which  show  a  preponderance  of  the  manic- 
depressive  psychosis,  as  there  are  families  that  show  a  preponderance 
of  the  precox  type  of  reaction. 

In  harmony  with  the  hereditary  tendencies  which  appear  to  be 
present  in  this  group  of  cases  it  is  found  that  the  group  may  be  widely 
differentiated  into  two  extremes,  the  one  in  which  the  constitutional 
factors  appear  to  be  predominantly  in  evidence  and  in  which  the 
various  attacks  appear  to  originate  either  without  any  cause  at  all 
or  at  least  without  a  determinable  or  apparent  cause  that  is  sufficient. 
On  the  other  hand  there  is  a  group  which  appears  to  be  more  or  less 
largely  determined  by  causes  which  are  apparent  such  as  the  inability 
of  the  individual  to  adjust  to  certain  conditions  of  life,  and  repeated 
breakdowns  with  the  return  of  these  conditions.  It  is  important  to 
recognize  these  two  groups  of  cases  between  which  of  course  every 
intermediate  variety  may  be  found,  because  of  the  significant  bearing 
which  the  type  of  etiological  factor  has  upon  the  probable  outcome 
of  the  therapeutic  attack. 

In  describing  this  type  of  mental  disturbance  the  two  principal 


634 


MANIC-DEPRESSIVE  PSYCHOSIS 


phases — the  manic  and  the  depressive — will  first  be  described  and 
then  the  various  forms  of  periodic  psychosis  of  the  manic-depressive 
group  will  receive  consideration,  while  finally  certain  less  common 
combinations  of  symptoms  known  as  the  mixed  states  will  receive 
attention. 

Manic  Phase. — The  cardinal  symptoms  of  the  manic  phase  of  manic- 
depressive  psychosis  are  three  in  number,  namely  (1)  flight  of  ideas, 
(2)  -psycJiomotor  hyperactivity,  (3)  emotional  exaltation.  These  three 
symptoms  may  manifest  themselves  with  any  degree  of  severity,  and 
the  severity  of  the  symptoms  may  vary  within  wide  limits  at  different 
times  throughout  the  course  of  the  attack.  The  three  symptoms,  too, 
may  not  be  all  of  the  same  degree  of  severity,  for  example,  the  flight 

of  ideas  may  be  extreme  and  out 
of  proportion  to  the  degree  of 
psychomotor  activity  which  may 
show  only  a  slight  increase. 

Taking  the  attack  as  a  whole 
the  ordinary  acute  varieties  are 
generally  designated  as  acute 
mania  or  acute  maniacal  excite- 
ment. Still  milder  grades  are 
spoken  of  as  hypomania  and  the 
more  severe  grades  are  generally 
termed  acute  delirious  mania. 
These  three  degrees  of  excitement 
are  the  most  convenient  captions 
under  which  to  describe  the 
manic  phase. 

Hypoviania,  which  is  the  mild- 
est of  these  three  degrees,  merges 
of  course,  upon  the  one  hand  into 
higher  grades  of  excitement,  such 
as  the  acute  maniacal,  and  on  the 
other  into  conditions  of  cyclothy- 
mia, which  may  be  termed  hypomaniacal,  but  which  constitute  a  special 
group  of  this  class  of  cases  which  will  be  considered  separately.  In 
this  condition,  at  least  in  the  simpler  cases,  there  is  a  disorder  which 
involves  the  process  of  thinking  more  than  it  does  the  content  of 
thought.  The  particular  ideas  and  acts  may  not  be  unusual  and  yet 
when  taken  together  in  association  with  each  other  are  seen  to  be 
abnormal.  This  state  of  affairs  is  excellently  illustrated  by  the  hypo- 
thetical case  cited  by  Mercier  ■}  "  Its  subject  rises  early,  full  of  schemes 
of  business  or  pleasure.  He  fusses  noisily  about  the  house,  indifferent 
to  his  disturbance  of  other  people's  slumbers.  He  is  very  impatient 
of  delay,  he  cannot  wait  a  minute  for  anything  that  he  wants,  and  if 


Fig.  302. — Facial  expression  of  hypomanic 
with  flight  of  ideas. 


>  A  Text-book  of  Insanity,  The  Macmillan  Co.,  1914. 


MANIC  PHASE  '       635 

it  is  not  forthcoming  on  the  instant,  he  jflies  into  a  rage.  The  course 
of  the  post  is  not  expeditious  enough  for  him.  He  sends  his  letters 
by  telegraph,  and  his  letters  are  extraordinarily  numerous.  They 
would  be  numerous  in  any  case,  but  their  number  is  doubled,  and 
more  than  doubled,  by  the  frequent  changes  of  his  mind,  and  by  the 
impulsiveness  with. which  he  acts  upon  every  passing  whim.  He 
determines  to  make  some  purchase,  probably  a  very  unnecessary  one, 
but  one  for  which  he  can  adduce  twenty  plausible  reasons,  and  he 
writes  to  tell  his  solicitor  that  he  will  call  the  next  morning.  Scarcely 
is  the  letter  posted  when  he  sees  that  he  will  attain  his  object  more 
quickly  by  asking  his  solicitor  to  lunch.  He  telegraphs  accordingly. 
Before  his  messenger  returns,  it  occurs  to  him  that  he  had  better  ask 
the  vendor  to  lunch  also.  Another  telegram  is  dispatched,  and  since 
he  cannot  entertain  more  than  one  visitor  at  his  club,  another  must 
be  sent  to  the  solicitor  to  announce  the  change  to  a  hotel.  Then  he 
remembers  that  he  has  been  drawing  heavily  of  late  on  his  banking 
account,  and  that  he  may  not  have  the  necessary  funds  available. 
Another  telegram  to  the  bank.  But  if  there  are  insufficient  funds  in 
the  bank,  he  will  have  to  sell  stock  to  raise  the  funds;  another  telegram 
to  his  broker.  Then  he  determines  that  it  will  be  better  to  pledge  the 
stock  to  the  bank  rather  than  to  sell  it.  More  telegrams  to  the  broker 
and  to  the  bank.  The  broker  won't  like  the  contradictory  orders — 
never  mind;  ask  him  to  dinner — ask  them  all  to  dinner.  Put  off  the 
lunch  and  have  a  dinner  instead,  and  ask  the  solicitor,  the  vendor, 
the  banker  and  the  broker.  Yes,  and  why  not  Smith  and  Jones  and 
Robinson  as  well?  More  telegrams;  and  then,  since  two  out  of  three 
of  the  invited  guests  decline,  the  whole  thing  is  postponed,  also  by 
telegraph.  Meantime,  in  the  intervals  of  telegraphing,  his  hands 
have  been  full.  He  has  been  constantly  ringing  the  bell  and  giving 
orders — giving  them,  modifying  them,  and  countermanding  them — 
constantly  wanting  something  fresh,  running  up  and  down  stairs, 
writing  letters,  haranguing  this  person  and  that,  flying  into  a  rage 
upon  the  slightest  opposition,  tearing  the  bell  down  on  the  slightest 
delay,  and  talking  almost  incessantly." 

In  this  example  the  subject's  acts  might,  almost  all  of  them,  be 
considered  normal,  with  of  course  the  exception  of  those  due  to  undue 
irritability  or  anger.  Aside  from  this,  however,  each  act  is  consis- 
tently directed  to  some  definite  aim.  The  disorder  is  not  in  the  con- 
tent of  thought  so  much  as  in  the  process  of  thinking,  and  manifests 
itself  by  a  rapid  and  too  frequent  change  of  direction.  This  is  the 
phenomenon  known  as  fligJit  of  ideas,  and  may  be  perhaps  better 
illustrated  by  a  stenogram: 

"Do  you  know  I  was  kidnapped  to  be  sent  here  twice.  I  saw  a 
mock  funeral  of  me  before  I  left  home.  This  was  done  because  I  am 
a  great  inventor.  The  pope  of  Rome  is  the  greatest  human  being  in 
the  universe.  He  is  the  head  of  the  Catholic  Church.  My  head 
(association  of  the  word  head  in  two  different  meanings)  is  good  and 


636  MANIC-DEPRESSIVE  PSYCHOSIS 

sound,  and  I  am  certainly  not  insane.  Do  you  hear  the  ticking  of 
the  clock?  (External  association.)  It  says,  'call  the  little  heifer,  the 
heifer  is  sick.'  Did  you  ever  see  the  gloves  veterinary  surgeons  use 
when  they  doctor  sick  cows?  (Internal  association.)  Say!  what  are 
you  keeping  me  here  for  anyhow?  I  want  to  go  home.  (Here  he  was 
asked  how  he  slept  at  night.)  I  have  slept  excellently;  that  is  because 
I  am  of  such  a  strong  constitution.  The  Constitution  of  the  United 
States  (association  as  above  with  the  word  head — probably  the  asso- 
ciation is  in  large  part  at  least  a  sound  or,  as  it  is  called,  a  clang 
association)  was  signed  by  Thomas  Jefferson.  He  was  just  a  man, 
but  he  was  not  the  inventor  I  am." 

In  this  phenomenon  of  flight  of  ideas  the  patient  either  has  no  guid- 
ing idea  or  else  at  once  loses  it  so  that  there  is  no  consistent  and  sus- 
tained effort  directed  toward  attaining  a  goal  idea,  and  the  thought 
therefore  wanders  here  and  there  under  the  influence  of  chance 
associations.  As  a  result  the  train  of  thought  instead  of  progressing, 
changes  direction,  frequently  returns  upon  itself,  and  never  reaches 
a  logical  end.  The  various  ideas  are  not  on  that  account,  however, 
incoherent,  that  is,  they  do  not  fail  to  cohere  or  to  be  connected  with 
one  another,  although  it  may  be  quite  impossible  at  times  to  see 
wherein  this  connection  lies.  If  the  associations  are  external,  that  is, 
originate  in  the  surroundings,  it  is  often  quite  possible  to  place  them; 
when,  however,  they  are  internal,  that  is,  originate  within  the  patient's 
mind,  it  maj^  be  quite  impossible  to  conceive  what  they  are.  In  the 
example  just  cited,  while  there  are  many  places  where  the  connecting 
link  is  missing,  probably  because  it  was  an  association  formed  entirely 
within  the  patient's  mind,  still  the  connection  can  be  made  out  in  a 
sufficient  number  of  instances  to  establish  the  characteristics  of  the 
train  of  thought.  One  of  the  principal  characteristics  of  this  type 
of  the  train  of  thought  is,  as  we  have  seen,  its  great  tendency  to  change 
of  direction,  and  when,  for  example,  this  change  of  direction  takes 
place  under  the  influence  of  external  associations,  such,  for  example, 
as  the  ticking  of  the  clock,  as  noted  in  the  stenogram  given,  the 
phenomenon  is  known  as  distractibility.  Any  sensory  impression  is 
liable  to  be  the  starting-point  of  idea  association,  so  that  these  patients' 
trains  of  thought  may  be  turned  at  will,  almost,  by  such  devices  as 
shaking  a  bunch  of  keys  before  them,  saying  some  word  loudly,  show- 
ing them  a  newspaper,  or  in  other  words  momentarily  distracting  their 
attention. 

The  emotional  exaltation  is  also  well  shown  in  Mercier's  example. 
The  patient  is  constantly  doing  things  which  testify  to  his  idea  of  his 
own  importance.  One  is  struck,  however,  by  a  symptom  in  the 
emotional  field  which  is  perhaps  more  fundamental  than  the  simple 
exaltation  and  which  corresponds  to  the  symptom  just  cited  and  the 
motility  disorders  to  be  described,  that  is,  the  great  lability  of  the 
emotions,  the  rapid  play  of  different  emotional  reactions,  exaltation 
giving  place  to  irritability,  to  anger,  annoyance,  and  the  like.    There 


MANIC  PHASE  637 

is  no  sustained  emotional  attitude,  as  there  is  no  sustained  direction 
in  the  train  of  thought.  The  psychomotor  hyperactivity  is  also  well 
illustrated  by  this  case.  The  activity  of  the  patient  is  seen  to  be  con- 
stant and  unremitting,  but  again  it  has  the  same  qualities  as  the  train 
of  thought  and  the  emotional  attitudes  inasmuch  as  it  is  not  sustained 
for  any  length  of  time  in  any  particular  direction,  it  does  not  get 
anywhere,  while  from  time  to  time,  under  the  influence  of  rapid 
changes  of  emotion  the  acts  tend  to  impulsiveness.  There  is  marked 
pressure  of  activity  just  as  there  is  pressure  of  speech,  and  the  patient 
appears  to  be  living  under  terrific  and  unremitting  tension  without 
power  of  direction. 

In  this  condition,  therefore,  the  patient  is  constantly  active,  busying 
himself  about  one  thing  and  another,  talking  continuously  meanwhile, 
often  in  a  loud  and  rather  boisterous  manner,  while  emotionally, 
exaltation  is  manifested  by  good  humor,  a  smiling  countenance  and 
increased  self-esteem,  punctuated  mayhap  by  attacks  of  irritability 
or  impulsive  anger  from  little  or  no  cause.  His  confidence  in  his  own 
ability  is  unqualified  and  is  shown  in  the  outlining  of  all  manner  of 
schemes  of  work,  investments,  business  enterprises  and  the  like.  Flight 
of  ideas  is  marked,  though  not  of  high  degree,  the  conversation  changing 
at  frequent  intervals  from  subject  to  subject  and  the  activities  show 
a  like  characteristic,  there  being  no  consistent  effort  directed  at  any 
one  aim  for  any  length  of  time.  Letters  are  often  written  in  great 
numbers  and  their  contents  exhibit  the  same  characteristics  as  do  the 
speech  and  conduct.  The  patient  is  fully  oriented,  there  is  no  cloud- 
ing of  consciousness  nor  delusions.  In  spite,  however,  of  the  lucidity 
and  apparent  abundance  of  energy  the  real  efficiency  of  the  indi- 
vidual is  greatly  reduced  because  of  the  lack  of  consecutiveness  in 
application. 

Ofttimes  the  picture  is  complicated  by  the  addition  of  symptoms 
due  to  alcoholic  indulgence  which  is  very  common  with  patients  in 
this  condition,  many  of  whom  show  marked  moral  delinquencies,  but 
because  of  their  lucidity  and  facility  of  expression  often  elude  the 
authorities,  being  at  once  discharged  after  examination  when  appre- 
hended because  of  supposed  mental  disorder.  This  complication 
with  alcohol  will  be  spoken  of  again  under  the  head  of  Diagnosis. 
Sexual  excitement  is  also  quite  frequently  and  characteristically  in 
evidence  in  these  cases  and  leads  to  moral  delinquencies  which  show  a 
still  further  departure  from  the  patient's  normal  manner  of  conducting 
himself.  When  complicated  with  alcohol  the  whole  conduct  may  be 
quite  non-understandable  except  to  those  trained  in  the  recognition  of 
this  class  of  cases.  The  sexual  excitement,  of  course,  is  an  especially 
unfortunate  and  dangerous  symptom  in  young  women  and  may  lead 
to  particularly  regrettable  actions. 

Acute  Mania. — ^This  next  grade  of  maniacal  excitement  presents 
perhaps  the  most  characteristic  picture  of  this  phase  of  the  disease, 
exhibiting  the  symptoms  to  best  advantage,  though  it  must  be  under- 


638  MANIC-DEPRESSIVE  PSYCHOSIS 

stood  that  the  symptoms  of  the  different  grades  differ  only  in  degree, 
intermingle  and  are  found  alike  in  all  the  conditions. 

In  this  degree  of  excitement  the  flight  of  ideas  is  well  marked  and 
may  even  become  so  extreme  at  times  that  the  train  of  thought  has 
the  appearance  of  being  quite  incoherent.  Distractibility  is  a  prom- 
inent feature  and  the  patients  are  constantly  diverted  by  inconse- 
quential happenings  in  their  environment.  The  tendency  to  rhyme  is 
quite  frequent  and  the  words  heard  by  the  patient  spoken  by  those 
about  him,  although  they  may  have  no  reference  to  him  or  be  addressed 
to  him  in  any  way  are  often  woven  into  or  form  the  starting-point  for 
these  rhymes  or  for  associations.  The  characteristic  of  these  associa- 
tions is  their  superficiality  and  when  words  that  are  heard  are  intro- 
duced into  their  conversation  the  basis  of  their  choice  is  often  nothing 
more  than  the  sound  similarity  (clang  association) .  It  is  quite  remark- 
able how  such  a  patient  who  is  apparently  paying  no  heed  to  what  is 
going  on  about  him  will  catch  a  chance  word  or  phrase  uttered  by  some 
one,  perhaps  a  considerable  distance  away,  and  introduce  it  into  the 
stream  of  his  conversation.  Consciousness  may  be  somewhat  clouded 
and  there  may  be  at  least  apparent  disorientation,  particularly  for 
persons.  This  apparent  personal  disorientation,  however,  is  dependent 
in  the  main  upon  two  factors.  In  the  first  instance  the  patient  does  not 
adequately  perceive  the  environment,  he  does  not  dwell  long  enough 
upon  any  one  particular  element  of  it  to  comprehensively  grasp  it  in  the 
rapid  and  transitory  survey  which  it  receives  from  him;  its  elements 
are  not  adequately  perceived  and  therefore  are  often  misunderstood, 
partly  because  of  this  superficial  attitude  toward  the  environment 
and  partly,  also  probably,  because  of  deeper  reasons.  Slight  resem- 
blances to  friends  or  relatives  are  often  seen  in  the  patients  and 
nurses,  and  these  resemblances  are  magnified  out  of  due  proportion, 
and  so  these  various  persons  are  addressed  by  the  names  of  members 
of  the  patient's  family  for  instance.  These  resemblances  do  not 
necessarily  result  in  a  permanent  and  fixed  mistake.  The  person  who 
is  at  one  moment  addressed  by  one  name  is  a  little  later  addressed 
by  another,  and  not  infrequently  the  whole  situation  is  further  com- 
plicated by  the  wit  reaction  of  the  patient  who  gets  a  good  deal  of 
fun  out  of  his  facetious  remarks  and  his  apparently  meaningless 
mistakes.  These  errors  being  not  firmly  fixed  are  frequently  sponta- 
neously corrected  by  the  patient,  at  least  at  times. 

The  disorders  of  attention,  flight  of  ideas,  and  distractibility  are 
all  elements  which  produce  a  transitory  and  a  superficial  survey  of  the 
environment  by  prohibiting  any  fixation  or  dwelling  upon  any  particu- 
lar element  of  the  environment  or  even  of  the  ego  and  tend  to  produce 
a  condition  of  the  content  of  consciousness  in  which  all  of  the  ideas 
are  given  the  same  value.  No  one  thing  is  attended  to  long  enough  to 
enhance  its  importance  over  that  of  others.  The  patient  voices  ideas 
first  about  this  subject  and  then  about  that,  changing  from  one  to  the 
other,  not  because  of  any  appreciation  of  differences  of  importance, 


MANIC  PHASE  639 

but  in  response  to  the  pressure  which  makes  it  impossible  for  him  to 
rest  anywhere,  so  that  all  ideas  tend  to  reach  the  same  level  of  impor- 
tance in  his  consciousness.    There  is  what  is  called  a  leveling  of  ideas. 

While  hallucinations  are  not  an  essential  part  of  the  picture  they 
may  occur,  but  when  they  do,  like  all  of  the  other  elements,  they  tend 
to  be  only  transitory  and  usually  are  rather  simple  and  elementary 
in  character. 

The  delusions  also  are  inclined  to  be  changeable.  They  partake 
characteristically,  when  present,  of  the  grandiose  character,  but  usually 
lack  the  element  of  extreme  improbability  found  in  conditions  of 
dementia  precox  and  general  paresis.  Occasionally  we  see  a  persecu- 
tory paranoid  system  of  delusions  develop  in  the  manic  phase  of  this 
disease,  but  this  class  of  delusions  is  more  apt  to  develop  and  present 
a  fairly  well-organized  system  in  the  milder  grades  of  excitement. 

The  psychomotor  activity  is  constant.  The  patients  are  unable  to 
remain  at  rest  (pressure  of  activity),  they  run  and  jump  and  turn 
somersaults,  wave  the  arms  about,  tear  up  clothing,  destroy  plants, 
break  furniture,  howl  and  yell  all  night  long,  and  go  almost  absolutely 
without  sleep.  Sexual  excitement  may  be  prominently  in  evidence. 
The  excitement  may  be  so  great  that  the  patient  does  not  even  take 
time  to  eat;  food  placed  before  him  is  perhaps  tasted  and  then  thrown 
about  like  everything  else  that  comes  in  his  way,  so  that  with  the  lack 
of  nutrition,  lack  of  sleep,  and  with  the  unremitting  activity,  emacia- 
tion is  a  constant  feature.  In  less-marked  degrees  of  excitement, 
however,  where  the  feeling  of  well-being  is  the  controlling  factor,  it  is 
common  for  the  patient  to  gain  somewhat  in  weight. 

The  emotional  exaltation  is  marked  and  shown  by  boisterous  laughter 
and  remarks  showing  exaggerated  ideas  of  self-esteem.  Patients, 
however,  are  spasmodically  apt  to  be  irritable,  bursting  into  attacks  of 
anger  without  adequate  reason  and  often  are  a  constant  source  of 
trouble,  annoyance  and  agitation  upon  the  wards  where  they  are 
confined.  The  emotional  condition  is  as  changeable  as  the  trend  of 
thought  or  of  the  direction  of  the  activities,  and  emotions  of  radically 
opposed  qualities  may  easily  replace  each  other. 

Delirious  Mania. — This  condition  is  merely  an  aggravated  state  of 
the  acute  mania  already  described.  The  flight  of  ideas  here  has  pro- 
ceeded to  almost  complete  incoherence.  The  activity  is  unremitting 
and  consciousness  is  more  clouded  and  hallucinations  more  in  evidence. 
The  lack  of  sleep  and  proper  nourishment,  with  rapid  emaciation,  soon 
leads  to  great  physical  exhaustion,  while  the  constant  activity  not 
unusually  results  in  slight  wounds  which,  even  though  properly 
dressed,  are  soon  exposed  to  infection  when  the  patient  tears  off  the 
dressing.  Local  areas  of  suppuration  thus  develop,  there  is  a  mild 
degree  of  infection  with  some  temperature,  which  coupled  with  the  ex- 
haustion and  the  toxemia,  produce  a  picture  more  distinctly  delirious 
with  marked  clouding  of  consciousness  and  great  incoherence. 

The  acute  delirious  mania  which  used  to  be  described  and  was 


640  MANIC-DEPRESSIVE  PSYCHOSIS 

regarded  as  always  fatal,  was  undoubtedly  in  a  certain  proportion  of 
cases  the  hypomania  of  the  manic-depressive  psychosis  to  which 
perhaps  had  been  added,  as  just  described,  symptoms  of  infection, 
toxemia,  and  exhaustion,  which  made  the  picture  one  of  delirium. 
Many  other  cases  were  undoubtedly  also  included  in  this  general 
caption,  more  particularly  deliria  associated  with  acute  diseases  of 
the  internal  organs,  such  as  acute  nephritis  or  pneumonia.  One  who 
has  had  experience  with  these  cases  can  understand  how  such  con- 
ditions might  go  unrecognized,  owing  to  the  almost  physical  impossi- 
bility of  subjecting  such  patients  in  their  wildly  excited  condition  to 
anything  approaching  an  adequate  physical  examination. 

Chronic  Mania. — There  are  a  very  few  cases  that  pass  into  a  con- 
dition of  chronic  mania  and  usually,  though  not  always,  have  mild 
excitements  that  may  last  for  a  number  of  years.  These  conditions, 
on  the  other  hand,  may  be  practically  nothing  else  but  character 
anomalies.  Such  prolonged  phases  of  the  disease,  however,  must  be 
borne  in  mind  as  possibilities. 

Depressive  Phase. — ^Like  the  manic  phase  this  phase  also  manifests 
itself  by  three  cardinal  symptoms  each  diametrically  opposed  to  the 
corresponding  symptom  of  the  manic  phase,  namely  (1)  difficulty  of 
thinking,  (2)  psychomotor  retardation,  (3)  emotional  depression. 

This  group  of  symptoms  may,  as  with  the  manic  group,  manifest 
itself  with  any  degree  of  severity,  and  the  three  symptoms  may  sever- 
ally and  individually  vary,  irrespective  of  each  other.  The  retardation, 
for  example,  may  be  quite  out  of  all  proportion  to  the  depression. 

As  with  the  manic  phase,  it  is  convenient  to  consider  the  depressive 
phase  in  three  different  grades. 

Simple  Retardation. — The  word  retardation  is  here  used  to  refer  not 
only  to  to  psychomotor  retardation,  but  to  the  difficulty  of  thinking 
also,  probably  quite  similar  phenomena,  the  one  more  particularly  in 
the  sphere  of  thought  and  the  other  more  particularly  in  the  sphere 
of  psychomotility.  These  patients  move  and  speak  slowly  and  per- 
haps in  a  low  voice,  by  preference  answering  questions  in  monosyl- 
lables. These  outward  evidences  of  difficulty  of  thinking  and  moving 
are,  however,  more  marked  in  the  next  stage  of  the  depressive  phase, 
that  is,  in  acute  melancholia,  while  here  it  is  more  usual  to  see  the 
patients  merely  preferring  to  be  by  themselves,  disinclined  to  associate 
with  others,  keeping  to  their  room,  and  quite  unable  to  make  any 
mental  effort.  They  are  not  equal  at  all  to  going  on  with  their  work. 
They  may  not,  for  example,  feel  equal  to  writing  letters  or  even  to 
reading  the  newspaper. 

Emotionally  these  patients  are  usually  somewhat  depressed,  but  the 
depression  may  not  be  especially  marked,  it  may  only  appear  on 
questioning.  Consciousness  is  clear  and  the  patients  are  fully  oriented 
and  often  have  a  realization  of  their  mental  invalidism. 

Acute  Melancholia. — In  this  grade  of  depression  the  three  cardinal 
symptoms  are  manifested  in  a  much  more  pronounced  way.     The 


DEPRESSIVE  PHASE 


641 


Fig.  303.— Severe  de- 
pression of  several  years' 
duration. 


patients  are  characteristically  inactive,  sitting  by  themselves,  showing 
little  or  no  tendency  to  associate  with  others,  their  movements  are 
slow  and  deliberate  (executive  retardation)  and  it  often  takes  a  con- 
siderable time  to  initiate  them  (initial  re- 
tardation) .  The  speech  is  similarly  affected ; 
it  is  slow,  often  monosyllabic,  and  sometimes 
almost  inaudible.  Initial  retardation  is 
noticeable  here  also.  The  emotional  depres- 
sion is  profound  and  is  indicated  in  the  general 
attitude  of  the  patient  which  is  one  of  flexion 
of  the  body,  the  hands  lying  limp  in  the  lap, 
the  head  inclined  forward,  the  chin  resting 
on  the  breast,  and  a  marked  facial  expression 
of  sadness.  The  subjective  state  of  these 
patients  is  described  by  a  feeling  of  difficulty 
of  thinking  and  grasping  the  meanings  of 
things  and  of  their  feeling  of  inadequacy,  of 
incapacity  for  all  effort,  or  even  thought. 
There  is  a  marked  feeling  of  decrease  in  the 
mental  activities,  and  the  patient  does  not 
feel  that  he  has  control  of  his  mind  and  can 
use  it  effectively.  In  the  same  way  he  feels 
an  interference  when  he  comes  to  exert  his 
will  in  the  performance  of  voluntary  acts. 
There  is  a  lack  of  energy,  lack  of  ability  to 

initiate  or  to  sustain  an  act  or  a  series  of  acts,  and  in  the  mental 
sphere  alone  the  patient  finds  himself  quite  unable  to  carry  out  a 
series  of  consecutive  mental  acts  which  lead  to  a  logical  issue.  He 
cannot  come  to  conclusions,  he  has  an  overwhelming  sense  of  weari- 
ness, of  relaxation,  of  inadequacy. 

This  general  feeling  of  inadequacy  and  difficulty 

of  thinking  as  above  described  fits  into  and  forms 

a  part  of  the  emotional  attitude  and  acts  with 

it  in  determining  the  character  of  the  delusions. 

The  delusions   are  typically  self-accusatory   and 

hypochondriacal.     The  patients  think  themselves 

responsible  for  all  the  sin,  wickedness,  privation 

and  suffering  in  the  world;  they  are  the  cause  of 

the  unfortunate  condition  of  their  fellow  patients; 

they  themselves  have  committed  some  great  sin 

and  their  souls  are  forever  lost.    As  they  occupy 

themselves  with  their  own  moral  states  so  they 

occupy  themselves   with  their  bodily  condition 

and  believe  themselves  sufferers  from  incurable 

disease,  think  that  their  organs  are  decayed,  something  has  happened 

to  their  brains,  their  bowels  are  stopped  up,  their  bones  broken,  and 

other  such  somatopsychic  ideas.     When  the  organic  sensations  are 

41 


Fig.  304. — Depressive 
facias. 


642  MANIC-DEPRESSIVE  PSYCHOSIS 

altered  patients  have  strange  feelings  which  they  interpret  as  indicat- 
ing some  mysterious  thing  going  on  within  their  body,  and  such  sensa- 
tions may  be  at  the  basis  or  associated  with  some  of  the  hypochon- 
driacal ideas.  The  emotional  depression  may  at  times  reach  a  very 
high  grade  and  express  itself  in  anxiety  attacks,  moods  of  apprehension, 
fear  of  impending  danger,  a  nameless  dread  of  something  going  to 
happen,  and  the  like.  The  whole  world  is  looked  at,  so  to  speak, 
through  blue  glasses.  The  sad,  depressed  mood  colors  every  percep- 
tion, and  so  the  perceptions  are  more  or  less  incorrect  and  distorted  to 
fit  the  mood. 

Hallucinations  may  occur,  but  consciousness  is  usually  clear  and 
the  patient  well  oriented.  There  may,  however,  be  a  lack  of  orienta- 
tion toward  their  surroundings  dependent  upon  the  fact  that  they 
are  wrapped  up  in  their  own  thoughts  and  the  environment  is  not 
attended  to. 

Physically  there  is  almost  always  constipation,  a  coated  tongue, 
indicanuria,  poor  appetite,  loss  of  weight,  disturbed  sleep,  and  often 
circulatory  disturbances  with  cold  extremities. 

Depressive  Stupor. — This  is  the  third  and  most  severe  grade  of  the 
depressive  phase.  In  this  condition  the  retardation,  both  in  the  field 
of  psychomotility,  and  in  that  of  thought,  has  proceeded  to  the  extent 
that  the  patient  lies  wholly  inactive  and  mute;  he  has  to  be  tube  fed, 
and  his  every  want  ministered  to. 

During  this  period  of  absolute  inactivity  it  may  be  that  the  patient 
is  suffering  from  delusions  and  hallucinations  of  a  depressive  and 
horrifying  nature  which  perhaps  are  shadowed  forth  by  an  anxious 
expression  of  countenance,  but  the  details  of  which  can  only  be  learned 
after  the  patient  has  aroused  suflBciently  from  his  stupor  to  be  able  to 
express  himself.  The  hallucinations  may  appear  to  the  patient  much 
as  in  a  dream  and  absorb  his  attention  to  a  very  great  extent.  This 
condition  of  stupor  is  not  uncommon  in  the  course  of  the  depressive 
phase,  but  usually  occurs  as  an  episode  rather  than  as  a  distinct  form 
of  the  disease. 

Chronic  Depression. — There  are  certain  patients  who  present  for 
long  periods  of  time  a  depressed  mood.  These  cases  may  be  mild 
depressive  phases  of  manic-depressive  psychosis  or  they  may  be 
character  anomalies  and  so  show  the  close  interrelations  between  the 
normal  fluctuations  of  emotion  and  those  that  are  pathological. 

The  Periodical  Types. — Under  this  head  are  included  those  forms  of 
the  manic-depressive  psychosis  which  from  time  to  time  have  been 
severally  described  as  recurrent  mania,  periodic  mania,  intermittent 
mania,  recurrent  melancholia,  insanity  of  double  form,  alternating 
insanity,  circular  insanity,  etc. 

All  of  these  psychoses  are  merely  different  manifestations  of  manic- 
depressive  psychosis,  the  manic  and  depressive  stages  being  represented 
in  various  relations,  often  separated  by  a  recovery  interval.  Thus  recur- 
rent mania  would  be  recurrent  attacks  of  a  manic  phase  separated  by 


THE  PERIODICAL  TYPES  643 

well  intervals,  similarly  for  recurrent  melancholia,  while  alternating 
insanity  would  consist  of  manic  and  depressive  attacks,  each  followed  by 
a  recovery  interval;  circular  insanity,  on  the  other  hand,  being  cycles 
of  manic  and  depressive  phases  without  intervals  of  separation,  while 
insanity  of  double  form  would  consist  of  cycles  of  excitation  and  de- 
pression, each  cycle  followed  by  a  lucid  interval.  Other  varieties  might 
be  described,  but  it  suffices  to  say  that  the  three  phases — manic,  de- 
pressive, and  lucid  interval — may  be  combined  in  any  possible  way,  and 
that  further  in  a  given  case  any  degree  of  the  manic  or  depressive  phase 
may  occur.  It  is  common,  too,  to  see  during  attacks  of  the  manic  phase 
transitory  attacks  of  depression,  while  during  the  depressive  phase  it 
is  equally  common  to  see  transitory  periods  of  euphoria. 

In  a  number  of  these  cases  the  attacks  reproduce  themselves  Qften 
at  very  definite  intervals  with  practically  photographic  accuracy  so 
that  the  patient  leads  a  life  the  events  of  which  can  be  predicted  with 
almost  absolute  precision.  Such  patients  not  infrequently  know  some 
little  time  beforehand  when  an  attack  is  coming  on,  and  the  physician 
may  be  able  to  see  the  approach  of  an  attack  the  moment  he  steps 
into  such  a  patient's  room  by  a  little  difference  in  the  arrangement  of 
things  that  indicates  the  way  matters  are  going. 

It  would  seem  that  the  patients  who  present  such  definite  cycles 
occurring  at  stated  intervals,  each  exactly  like  the  others,  belong  to 
the  group  of  cases  with  severe  constitutional  taint.  In  the  other  group 
of  cases,  that  group  in  which  external  conditions  seem  to  play  a  large 
part  in  the  etiology  of  the  several  attacks,  there  is  much  less  tendency 
to  regularity  in  their  occurrence,  and  as  heretofore  intimated,  much 
greater  hope  for  the  results  of  therapeutics. 

The  following  is  the  account  of  an  intelligent  woman  of  her  feelings 
in  both  periods  of  excitement  and  depression: 

"I  have  suffered  all  my  life  from  excitements  and  depressions, 
although  it  was  not  until  I  was  fifty-eight  years  of  age  that  my  family 
and  I  realized  I  was  really  mentally  sick,  and  required  institutional 
care.  During  youth  and  middle  age  my  excitements  were  of  a  mild 
character,  and  during  these  periods  I  considered  myself  normal.  I  felt 
peculiarly  happy  and  care-free.  I  managed  my  household  affairs  with 
the  greatest  ease.  I  entertained  and  mingled  in  society  with  pleasure 
and  zest.  I  was  lively,  talkative  and  I  have  reason  to  believe  I  was 
witty  and  entertaining.  I  could  work  without  an  effort.  I  at  times 
accomplished  almost  Herculean  tasks.  On  one  occasion  I  remember 
preparing  and  conducting  a  church  entertainment  by  which  the  sum  of 
$800  was  raised.  Of  late  years  my  excitements  have  grown  more  severe. 
I  begin  by  taking  an  overactive  interest  in  everything  going  on  around 
me.  Everything  seems  rosy.  I  feel  happy  and  nothing  depresses  me. 
I  feel  propelled  by  some  unknown  force  to  constant  action.  I  am  pos- 
sessed with  the  idea  of  righting  wrongs  and  straightening  out  things 
in  general.  All  the  faults  in  the  administration  of  the  ward,  the  hos- 
pital, and  the  government  must  be  corrected. 


644  MANIC-DEPRESSIVE  PSYCHOSIS 

"My  excitements  have  never  led  me  to  commit  any  acts  of  violence. 
I  occupy  myself  largely  in  talking  and  writing  letters.  My  room  is 
often  in  disorder  because  I  cannot  stay  at  one  job  long  enough  to  com- 
plete it.  As  I  feel  these  excitements  approaching,  I  request  the 
physician  in  charge  of  me  to  take  up  my  parole,  as  I  know  I  shall  be 
moved  to  do  and  say  many  foolish  things  of  which  I  will  be  ashamed 
later.  No  one  who  has  not  had  experience  can  realize  the  mortifica- 
tion of  having  been  insane. 

"My  depressions  in  early  life  were  as  mild  as  my  excitements,  the 
onset  was  gradual.  I  felt  a  disinclination  to  mingle  in  society.  When 
forced  to  do  so  I  sat  like  a  'dummy'  and  could  think  of  nothing  to 
say.  My  household  duties  became  a  burden.  One  after  another  of 
these  was  dropped  until  the  care  of  the  household  was  entirely  given 
over  to  relatives  or  servants.  I  learned  from  experience  a  treatment 
of  my  own.'  As  soon  as  I  felt  a  depression  approaching,  I  promptly 
dropped  everything  and  left  home  for  a  time.  I  found  by  getting 
away  from  family  cares  and  responsibilities,  and  from  the  demands 
of  society,  to  some  quiet  spot,  I  could  shorten  the  duration  of  these 
depressions.  In  recent  years  the  depressions  have  appeared  suddenly. 
One  day  I  went  to  town  to  do  some  shopping  for  a  friend.  I  went  to  a 
grocery  store  to  make  some  purchases.  It  suddenly  occurred  to  me 
that  I  could  make  these  to  much  better  advantage  at  the  market 
only  a  block  away.  Suddenly  I  realized  that  I  did  not  have  sufficient 
energy  to  go  to  the  market,  and  that  another  depression  was  upon 
me.  It  was  with  the  greatest  difficulty  that  I  ordered  the  goods, 
paid  for  them  and  came  home.  At  these  times  my  brain  feels  paralyzed. 
I  have  not  the  strength  or  ambition  to  do  anything.  I  am  apprehen- 
sive lest  some  harm  has  befallen  the  members  of  my  family,  but  to 
save  my  life,  I  could  not  write  or  telephone  to  find  out  if  my  fears 
are  true.  I  have  the  impulse  to  act,  but  it  seems  as  if  something 
shuts  down  and  prohibits  action.  I  see  my  clothes  becoming  soiled — 
I  know  I  should  change  them,  but  I  cannot  pull  out  the  drawer  of  my 
bureau  and  get  clean  ones.  This  inertia  is  greater  in  the  morning  than 
at  night.  Before  I  came  to  the  hospital  for  treatment  I  had  servants 
who  slept  at  home,  and  came  to  my  house  early  in  the  morning. 
When  my  husband  was  away  and  my  children  were  small,  it  devolved 
upon  me  to  admit  these  servants  early  in  the  morning.  I  knew  that 
when  morning  came  to  dress  and  go  down  stairs  would  be  impossible. 
I  solved  the  difficulty  by  dressing  the  night  before  and  sleeping  in  my 
clothes.  When  the  depression  is  most  profound,  I  move  in  a  fixed 
groove.  I  never  vary  a  hair's  breadth.  At  first  I  have  a  desire  to 
remain  in  bed.  Once  this  is  overcome  I  have  no  choice  but  to  remain 
up.  I  sit  in  the  same  seat  and  in  the  same  attitude  for  weeks.  As 
I  come  down  stairs  in  the  morning  I  am  apprehensive  lest  my  seat  be 
taken,  and  I  wonder  what  I  shall  do  if  it  should  be  occupied,  although 
the  sitting  room  is  well  supplied  with  comfortable  seats.  I  bring  a 
shawl  with  me,  and  place  it  in  the  chair  so  that  no  one  will  appropriate 
it  while  I  am  at  breakfast. 


THE  CYCLOTHYMIAS  645 

"  After  each  depression,  I  suffer  from  intense  pain  in  my  back,  side, 
shoulders  and  arms.  This  is  dull  and  aching  in  character,  and  remains 
with  me  for  weeks  after  the  depression  has  disappeared.  After  the 
last  depression  I  suffered  from  a  severe  attack  of  the  shingles.  The 
skin  eruption  has  now  disappeared,  but  the  pain  still  remains." 

The  Cyclothymias. — This  group  of  cases  presents  the  mildest  excite- 
ments and  depressions.  They  deviate  less  from  the  normal  than  the 
other  groups  and  are  only  considered  separately  because  of  their 
great  practical  importance.  They  are  quite  usually  not  recognized 
and  the  symptoms  are  attributed  to  all  sorts  of  things  other  than  the 
real  trouble.  It  must  not  be  lost  sight  of  in  considering  these  mild 
manic-depressive  fluctuations  that  a  slight  depression  may  recur  with- 
out the  psychosis  expressing  itself  by  a  fluctuation  to  the  opposite 
condition  of  excitement,  and  vice  versa.  So  that  the  picture  is  seen 
of  patients  presenting  from  time  to  time  mild  degrees  of  depression 
or  mild  degrees  of  excitement  without  anything  approaching  delu- 
sional formation  or  disorders  of  the  sensorium  and  therefore  attracting 
no  particular  attention  from  the  mental  side.  The  following  example 
illustrates  this  exceedingly  well:  He  is  a  man  who  devotes  himself 
largely  to  literary  work,  and  the  fluctuations  in  his  mental  state  are 
shown  excellently  well  by  his  ability  to  write.  The  onset  of  a  depres- 
sive phase  is  usually  shown  by  a  gradual,  though  more  or  less  rapid, 
falling  off  in  his  literary  ability.  He  is  first  unable  to  compose,  then 
he  gets  progressively  less  able  to  write  until  he  is  only  able  to  write 
the  simplest  things.  It  is  the  same  way  in  his  reading.  He  gravitates 
all  the  way  from  reading  connected  with  his  work  doM^n  through  the 
different  grades  of  literature  until  he  gets  to  fiction.  He  finally  finds 
himself  quite  incapacitated,  sitting  for  hours  gazing  out  of  the  window 
or  at  a  blank  wall,  and  while  rather  enjoying  company,  it  is  almost 
impossible  for  him  to  initiate  the  procedure  that  is  necessary  to  go 
anywhere.  He  finds  it  almost  impossible  to  dress,  to  get  out,  to  take 
the  cars,  and  the  like.  This  state  is  one  almost  entirely  of  retardation 
without  marked  emotional  depression.  During  the  opposite  condi- 
tion of  affairs  he  has  a  feeling  of  well-being  and  efficiency  in  marked 
contrast  to  his  feelings  during  the  depressive  period,  and  finds  himself 
quite  able  to  work  for  long  intervals  very  effectively. 

The  remarkable  transitions  from  phase  to  phase  are  shown  well  by 
one  of  his  experiences.  One  day,  having  been  writing  all  of  the  after- 
noon, he,  as  usual,  went  out  to  dinner,  leaving  his  papers  on  the  table, 
intending  to  resume  work  on  his  return.  When  he  came  back  and 
took  up  the  pen  to  write  he  found  that  the  incubus  of  his  depression 
was  upon  him.  He  had  difficulty  in  finding  words  and  finally  after 
two  hours'  effort  he  gave  it  up.  This  was  the  beginning  of  a  depression 
which  lasted  about  a  month.  During  this  time  he  constantly  tested 
his  ability  for  composition,  but  without  favorable  result.  Almost 
exactly  a  month  after  this  incident  he  undertook  to  answer  some 
personal  letters,  intending  to  write  only  short  letters  of  perhaps  three 


646  MANIC-DEPRESSIVE  PSYCHOSIS 

or  four  pages,  but  when  he  started  to  write  them  he  found  himself 
writing  easily  and  his  letters  spontaneously  expanded  to  eight  or  twelve 
pages  and  he  went  on  into  his  work  again. 

The  hyperthymic  types  show  exaggerated  activities  in  the  way  of 
the  usual  business  occupations,  writing  letters  and  the  like.  The 
judgment  is  apt  to  be  rather  poor  at  these  times  and  many  of  the 
business  ventures  come  to  grief,  though  not  necessarily  so.  Work  is 
easily  done,  without  having  rnade  effort,  and  the  patient  expends 
enormous  amounts  of  energy  over  long  periods  of  time.  Certain  types 
of  cases  are  meddlesome  and  troublesome,  tending  to  engage  in  dis- 
putes and  altercations,  and  to  bring  law  suits,  while  exaggerated 
eroticism  and  alcoholic  predilections  oftentimes  very  considerably 
color  the  picture. 

In  the  dysthymic  types  are  found  the  depressions  which  are  attributed 
in  large  part  to  neurasthenia  and  to  various  visceral  disturbances. 

These  cyclothymic  cases  not  infrequently  show  fluctuations  at 
periods  of  recurrent  physiological  activity  such  as  the  menstrual  period, 
while  it  must  never  be  lost  sight  of  that  not  a  few  so-called  dipso- 
manias are  really  recurrent  manic-depressive  attacks  in  which  the 
alcohol  is  resorted  to  shortly  after  the  attack  commences  and  then 
quite  usually  all  the  symptoms  from  which  the  patient  suffers  are 
attributed  to  the  alcoholic  indulgence.  It  is  important  to  bear  this 
class  of  cases  in  mind,  not  only  for  diagnostic  purposes,  but  in  order  that 
the  patient  should  be  dealt  with  fairly  as  a  sick  man. 

Perhaps  the  most  important  of  the  disturbances  in  this  group  of 
cases  are  the  visceral  disturbances.  There  are  a  large  number  of  con- 
ditions, particularly  the  false  gastropathies,  enteropathies,  cardi- 
opathies of  Dejerine,  etc.,  many  of  which  belong  here.  Inasmuch  as 
the  psychosis  is  not  recognized,  these  conditions  are  quite  naturally 
credited  with  being  the  cause  of  the  condition  of  the  patient.  Patients 
with  mild  depression  are  called  neurasthenic,  those  with  mild  excite- 
ment are  called  nervous,  and  the  accompanying  physical  condition  is 
credited  with  making  the  trouble.  The  patient  and  the  relatives 
consistently  take  this  attitude  and  the  physician  naturally  falls  into 
it.  No  one  wishes  to  acknowledge  the  possibility  of  a  mental  disorder, 
and  therefore  these  other  explanations  are  readily  accepted.  In  fact, 
the  condition  is  hardly  recognizable  at  its  true  value,  even  by  the 
practised  observer,  unless  a  full  account  of  the  patient's  life  is  available. 

After  a  while  the  symptoms  of  visceral  disturbance  clear  up  along 
with  the  disappearance  of  the  mental  symptoms,  and  the  change  is 
attributed  to  some  form  of  treatment,  a  special  dietary  regime,  or 
whatever  has  been  resorted  to  for  the  relief  of  the  symptoms.  Here  is  a 
group  of  cases  who  during  their  attacks  regularly  seek  the  specialist 
and  are  subjected  to  all  sorts  of  gastro-intestinal  treatment,  gastric 
lavage,  special  dietaries,  gynecological  manipulations  of  one  sort  and 
another,  metabolism  experiments,  treatment  for  auto-intoxication, 
eye-strain,  and  almost  everything  in  the  category  of  medical  specialism, 


THE  MIXED  STATES  647 

and  yet  characteristically  in  these  cases  nothing  is  found  in  the  physical 
condition  that  adequately  accounts  for  the  symptoms.  Another 
group  of  these  cases  are  the  paranoid  types.  These  patients  present 
typical  paranoid  symptoms  with  emotional  accompaniments  that  seem 
to  be  hardly  in  excess  of  what  is  demanded  as  normal  reaction  to  the 
delusional  state.  This  is  the  group  of  cases  that  has  given  origin  to 
a  great  deal  of  recent  discussion  with  regard  to  the  real  basis  of 
paranoia,  its  relations  to  manic-depressive  psychosis,  the  affective 
origin  of  paranoia,  and  its  basis  in  what  Specht  calls  the  "  affect  of 
suspiciousness." 

The  Mixed  States. — ^The  mixed  states  are  forms  of  manic-depressive 
psychosis  in  which  the  three  cardinal  symptoms  of  the  manic  and 
depressive  phases  are  mixed  so  that  the  resulting  state  is  neither  one. 
They  are:  (1)  maniacal  stupor,  (2)  agitated  depression,  (3)  unpro- 
ductive mania,  (4)  depressive  mania,  (5)  depression  with  flight  of 
ideas,  (6)  akinetic  mania.  It  will  suffice  to  merely  mention  the 
symptoms  of  these  groups. 

Maniacal  Stupor. — Emotional  exaltation,  decreased  psychomotor 
activity,  difficulty  of  thinking. 

Agitated  Depression. — Emotional  depression,  increased  psychomotor 
activity,  flight  of  ideas. 

Unproductive  Mania. — Emotional  exaltation,  increased  psychomotor 
activity,  difficulty  of  thinking. 

Depressive  Mania. — Emotional  depression,  difficulty  of  thinking, 
increased  psychomotor  activity. 

Depression  with  Flight  of  Ideas. — Emotional  depression,  flight  of 
ideas,  decreased  psychomotor  activity. 

Akinetic  Mania. — Emotional  exaltation,  flight  of  ideas,  decreased 
psychomotor  activity. 

Still  the  possibilities  are  not  exhausted.  It  is  quite  uncommon 
to  see  any  one  of  the  conditions  already  described  continue  pure  from 
the  commencement  to  the  end  of  the  attack.  In  the  manic  phase 
symptoms  of  depression  not  infrequently  crop  up  and  occupy  the  field 
temporarily,  while  during  the  depressive  phase  it  is  quite  as  common  to 
note  transitory  periods  of  excitement.  Then  it  is  quite  common  for 
manic  attacks  to  be  preceded  by  a  longer  or  shorter  attack  of  depres- 
sion, and  sometimes  such  a  period  of  depression  follows,  not  infre- 
quently but  partial  depression,  of  the  type  of  unproductive  mania. 
The  depressive  phase  shows  similar  variations,  more  particularly  it 
is  followed  by  a  short  period  of  exaltation.  Then,  again,  at  any 
stage  of  the  disease  a  mixed  state  may  crop  up  for  a  time,  so  that  we 
may  see  during  the  course  of  the  manic  phase  psychomotor  retardation 
occur  or  during  the  phase  of  depression  emotional  exaltation  may 
develop,  while  in  the  various  forms  of  the  periodic  psychoses  it  is  quite 
the  rule  to  find  these  mixed  states  at  the  transition  places  from  one 
phase  to  the  other,  all  of  the  symptoms  of  one  phase  not  equally  and 
contemporaneously   graduating  into   their   opposites.     Thus   during 


648  MANIC-DEPRESSIVE  PSYCHOSIS 

the  course  of  a  circular  psychosis  the  affect  may  change  from  depres- 
sion to  exaltation  before  the  psychomotor  retardation  has  given  place 
to  increased  psychomotor  activity,  thus  producing  a  temporary  mixed 
state. 

Involution  Melancholia. — The  group  of  cases  comprised  under  the 
term  involution  melancholia,  which  was  originally  used  by  Kraepelin, 
has  now  been  pretty  generally  conceded  to  belong  to  the  manic- 
depressive  group.  The  characteristics  of  the  disease  are  those  of  an 
anxious  depression  occurring  in  later  life.  The  group  is  such  a  con- 
siderable one  and  of  such  practical  importance,  however,  that  it  will 
be  specially  considered  along  with  other  depressions  of  later  life  in 
another  chapter.     (See  Chapter  XXIV.) 

Pathology. — There  are  no  specific  pathological  findings  in  this  psy- 
chosis, although  certain  degenerative  products  have  been  described 
in  cases  of  death  from  depressive  stupor.  Patients,  however,  char- 
acteristically recover  from  this  condition,  or  if  they  die  during  attacks, 
the  death  is  due  to  some  intercurrent  disease  which  itself  would  pro- 
duce changes  in  the  nervous  system  that  would  gradually  cloud  and 
perhaps  entirely  obscure  any  pathology  that  the  psychosis  might  have. 
A  condition  producing  death  itself  must  produce  serious  alterations  of 
the  central  nervous  system,  that  most  sensitive  of  reacting  portions  of 
the  human  body. 

Nature  of  Manic-depressive  Psychosis. — This  psychosis  perhaps,  as 
thoroughly  as  any  other,  has  withstood  throughout  the  years  any 
attempt  at  understanding  it,  while  as  opposed  to  dementia  precox 
the  symptoms  of  which  appear  quite  unpsychological,  the  symptoms 
of  the  manic-depressive  psychosis  in  either  one  of  its  phases,  more 
particularly  perhaps  in  the  depressive  phase,  are  quite  psychological, 
that  is,  quite  understandable.  The  patients,  to  begin  with,  present 
largely  normal  types  of  personality  before  the  advent  of  the  psychosis, 
and  during  the  symptoms  of  the  psychosis  they  ordinarily  are  not  so 
far  removed  in  their  conduct  or  in  the  character  of  their  ideas  as  to 
place  them,  so  to  speak,  in  a  class  by  themselves.  They  are  still  quite 
like  the  rest  of  us.  The  roots  of  the  psychosis  appear  to  spring  more 
distinctly  from  the  normal  life,  the  fluctuations  of  the  emotions  being 
quite  comparable  to  the  fluctuations  that  occur  in  everyone. 

However,  it  seemed  quite  impossible  to  understand  how  the  patients 
could  vary  from  one  extreme  to  its  diametrical  opposite  and  what  could 
possibly  be  the  explanation  of  such  shifts  of  position.  For  many  years, 
under  the  domination  of  Meynert,  the  changes  were  supposed  to  depend 
upon  changes  of  blood  supply,  upon  anemias  and  hyperemias.  When 
psychiatry,  however,  advanced  beyond  such  cross  types  of  explanation 
it  was  left  practically  without  anything  to  fall  back  upon.  Recently, 
however,  the  suggestive  work  of  Bleuler^  has  seemed  to  indicate  what 
may  at  bottom  be  the  true  explanation.    He  has  demonstrated  what 

1  The  Theory  of  Schizophrenic  Negativism,  Nervous  and  Mental  Disease  Monograph 
Series,  No.  11. 


NATURE  OF  MANIC-DEPRESSIVE  PSYCHOSIS  649 

he  calls  the  ambivalency  of  ideas.  This  ambivalency  gives,  as  he  under- 
stands it,  to  the  same  idea  two  contrary  feeling  tones  and  invests  the 
same  thought  simultaneously  with  a  positive  and  a  negative  character. 
Along  with  this  ambivalency  there  is  an  ambitendenc}'  which  sets 
free  w4th  every  tendency  a  counter-tendency.  With  this  basal  suppo- 
sition it  can  be  understood  why  the  fluctuation  of  the  manic-depres- 
sive is  a  fluctuation  between  conditions  which  are  diametrically 
opposed.  If  each  idea  has  associated  with  it  by  preference  the  idea 
which  is  absolutely  its  opposite,  if  each  feeling  has  associated  with  it 
by  preference  the  feeling  which  represents  its  exact  antithesis,  then 
there  is  reason  for  understanding  how  the  manic-depressive  gravitates 
between  these  two  conditions.  It  is  the  path  of  opposites  which 
is  met  with  at  every  turn  in  psychiatric  experiences.  Nothing 
suggests  white  more  easily  than  does  black,  nothing  suggests  love 
more  readily  than  hate.  The  opposed  ideas  and  feelings  stand  with 
relation  to  each  other  in  the  path  of  least  resistance,  and  when  one 
would  go  from  a  certain  idea  or  a  certain  feeling  in  any  direction  he 
finds  the  path  to  the  antithetical  idea  or  feeling  more  easily  passible 
than  the  path  to  any  other  goal. 

Assuming  the  hypothesis  of  ambivalency  and  ambitendency,  still 
what  is  the  explanation  of  the  afi^ect  fluctuations  in  this  psychosis? 
Here  as  elsewhere  in  the  mental  field  some  fundamental  psychic  con- 
flict undoubtedly  has  to  be  sought  to  which  the  patient  is  making 
efforts  of  adjustment.  This  is  precisely  the  starting-point  from  which, 
for  example,  dementia  precox  has  to  be  viewed.  But  here  are  individ- 
uals who  present  a  difi^erent  possibility  of  reaction,  a  different  reaction 
type  to  the  conflict  than  do  the  precox  patients.  This  statement,  of 
course,  must  not  be  taken  as  meaning  any  more  than  a  mere  putting 
into  words  of  what  is  found,  because  it  is  riot  understood  what  the 
differences  are  that  make  different  people  react  in  different  ways. 
Psychiatrists  are  only  upon  the  verge  of  being  able  to  ask  such  a 
question  intelligently.    They  are  not  yet  able  fully  to  answer  it. 

The  manic-depressive  psychosis,  however,  may  be  conceived  of  as 
an  eft'ort  at  compromise  and  at  defense,  resulting  from  an  endopsychic 
conflict.  In  the  depressive  phase  the  affect  has  broken  through  and 
invades  consciousness,  while  in  the  manic  phase  the  patient  by  feverish 
and  restless  activity,  by  a  constant  alertness,  fights  off  every  approach 
that  might  touch  him  on  a  painful  point,  that  might  reach  a  vulner- 
able spot.  It  would  seem  as  if  he  were  wildly  beating  about  to  keep 
off  all  intruders,  not  only  real  intruders,  but  all  possible,  prospective, 
or  thought-of  intruders.  And  so  the  manic  patient  is  already  quite 
inaccessible  and  all  of  his  reactions  are  especially  superficial,  as  witness 
the  word  associations  and  the  clang  associations.  He  moves  over  the 
surface,  which  he  endeavors  to  cover  completely  in  order  to  prevent 
penetration  at  any  point. 

This  is  only  a  general  statement  of  what  may  or  may  not  be  found 
by  further   study  to   be  the  case,  but   already  certain  indications 


650  MANIC-DEPRESSIVE  PSYCHOSIS 

are  known  that  give  some  hope  that  the  near  future  may  unravel  the 
meanings  of  manic-depressive  reaction.  Abraham^  particularly  has 
worked  with  this  group  and  lays  special  stress  upon  the  resemblance 
between  it  and  the  obsessional  neuroses.  The  relationship  to  hyper- 
thyroid  states  has  already  been  discussed  in  the  chapter  on  the  Internal 
Secretions.     (See  p.  147.) 

Course  and  Prognosis. — The  individual  attacks  vary  in  duration  from 
a  few  days  to  several  months.  Recovery  from  the  single  attack  is 
the  rule,  while  the  likelihood  of  subsequent  attacks  is  considerable. 
In  general,  therefore,  the  prognosis  is  good  for  the  separate  attacks 
and  is  rather  poor  as  to  ultimate  freedom  from  attacks.  As  pointed 
out  previously,  the  severe  constitutional  types  have  a  worse  prognosis 
than  those  types  in  which  the  etiological  factors  are  capable  of  removal. 
In  the  severe  constitutional  types  also  the  recurrent  attacks  tend  to 
repeat  with  photographic  accuracv  preceding  attacks,  while  in  the 
long  run  the  general  tendency  is  for  an  increase  in  the  length  of  attacks 
and  a  decrease  in  the  length  of  the  free  intervals. 

The  disease  pursues  its  course  without  any  special  tendency  to 
deterioration.  Although  mild  grades  of  dementia  have  been  described, 
terminating  protracted  attacks,  the  dementia  which  usually  super- 
venes in  the  course  of  the  disease  is  that  which  is  superadded  as  the 
result  of  the  changes  incident  to  arteriosclerosis  and  the  senium. 
Inasmuch  as  this  psychosis  tends  to  recur  throughout  life,  not  a  few 
of  the  patients  ultimately  reach  senescence. 

Differential  Diagnosis. — ^The  manic  phase  in  its  mildest  forms  is  often 
mistaken,  especially  where  it  leads  to  alcoholic  and  sexual  excesses, 
for  a  form  of  moral  obliquity.  In  the  somewhat  more  pronounced 
attacks  it  may  be  difficult  to  differentiate  it  from  other  excitements, 
more  particularlv  catatonic  excitement.  In  general  the  manic  excite- 
ment is  more  free  and  open,  there  is  less  tendency  to  constraint  along 
any  particular  line,  while  the  productivity  and  the  psychomotility  are 
not  as  meaningless  or  non-understandable  as  with  dementia  precox. 

The  depressive  phase  in  its  milder  manifestations  is  not  infrequently 
mistaken  for  neurasthenia,  and  in  its  more  pronounced  form  it  is 
extremely  difficult  to  differentiate  it  from  the  depression  of  dementia 
precox.  This  is  particularly  so  because  the  feeling  of  inadequacy 
of  the  manic-depressive  is  very  close  to  the  empty  feelings  with  loss 
of  affect  of  the  precox,  while  the  blocking  of  movement  and  expression 
in  the  latter  condition  outwardly  closely  resemble  the  retardation  in 
manic-depressive  psychosis.  The  stupor  of  catatonia  outwardly  also 
closely  resembles  the  manic-depressive  stupor,  except  that  it  is  more 
apt  to  be  associated  with  marked  negativism,  muscular  tension,  and 
perhaps  grimacing. 

The  greatest  difficulty,  as  between  manic-depressive  psychosis  and 

'  Psychoanalytic  Investigation  and  Treatment  of  Manic-depressive  Insanity  and 
Allied  Conditions,  Zentralblatt  fiir  Psychoanalyse,  a'oI.  ii,  No.  6;  abstracted  in  the 
Psychoanalytic  Review,  vol.  i,   No.  2. 


TREATMENT  651 

dementia  precox,  lies  with  the  differentiation  of  the  mixed  states.  Here 
the  resemblances  are  quite  close  and  prolonged  observation  is  often 
necessary  to  make  the  differentiation. 

It  must  not  be  forgotten  that  the  manic-depressive  psychosis  is  by 
no  means  a  clear-cut  definite  entity,  that  it  merges  in  all  directions 
into  other  conditions,  and  that  its  closest  affiliation  with  the  other 
psychosis  appears  on  the  surface  to  be  with  the  dementia  precox 
group.  There  are  quite  a  considerable  number  of  cases  in  which  a 
study  of  the  individual  attack  leaves  one  in  doubt  as  to  which  group  to 
place  the  patient  in,  manic  excitement,  for  example,  being  associated 
with  certain  catatoniform  symptoms,  catatonic  excitement  presenting 
a  fairly  typical  flight  of  ideas  and  the  like.  In  general  the  principle 
of  differentiation  is  first,  the  history  of  previous  attacks,  and  next  to 
this  history  of  repeated  attacks  is  a  history  of  attacks  of  both  manic 
and  depressive  character  occurring  in  the  individual  and  showing  no 
marked  tendency  to  deterioration.  Practically,  however,  it  is  quite 
impossible  to  make  a  differentiation  in  many  cases  and  patients  that 
are  at  one  time  placed  in  one  group,  for  example,  in  the  manic-depressive 
group,  are  found  later  to  probably  belong  in  the  precox  group,  because, 
for  example,  of  the  appearance  of  deterioration.  And  so  the  history 
of  the  diagnosis  in  these  two  groups  shows  a  constant  tendency  to 
fluctuation,  at  one  time  the  dementia  precox  group  being  enhanced  by 
cases  which  at  another  time  are  placed  in  the  manic-depressive  group. 
And  so  the  situation  fluctuates  back  and  forth,  the  best  evidence  of 
an  inherent  relationship  between  the  two. 

The  most  important  group  from  the  practical  standpoint  is  the  cyclo- 
thymic. These  patients  are  practically  always  wrongly  diagnosed  at 
first  and  often  over  and  over  again  for  considerable  periods  of  time. 
Most  generally,  as  already  mentioned,  they  fall  into  the  hands  of  the 
specialist  under  the  theory  of  some  disorder  of  the  internal  organs. 
When  an  attempt  is  made  to  really  understand  these  patients  one  is 
impressed  with  their  close  resemblances  to  the  obsessional  neurosis. 
It  may  be  quite  impossible,  at  least  by  any  means  other  than  a  very 
careful  and  detailed  study  of  the  patient,  to  differentiate  between  the 
two  conditions,  and  from  the  discussion  on  the  nature  of  the  manic- 
depressive  psychosis  it  will  be  seen  why  this  similarity  exists.  It  should 
be  borne  in  mind  also  that  it  is  not  difficult  to  confuse  mild  cyclothymic 
attacks  with  the  anxiety  neurosis. 

It  should,  of  course,  be  added  that  one  must  be  careful  and  not 
confuse  excitements  and  depressions  that  may  have  other  origins  as, 
for  example,  particularly  paresis,  the  symptomatic  and  toxic  psychoses. 

Treatment. — -There  has  been  the  general  feeling  in  years  past  about 
this  psychosis  that  the  attacks  were  self -limited.  This  has  probably 
been  dependent  to  no  small  extent  at  least  upon  the  extreme  regularity 
of  the  attacks  in  certain  patients.  These  patients,  however,  are  the 
patients  with  the  more  profound  constitutional  taints,  and  it  is  perhaps 
generally  true  that  in  this  class  of  patients  attacks  do  tend  to  run  a 


652  MANIC-DEPRESSIVE  PSYCHOSIS 

regular  course,  each  attack  being  approximately  of  the  same  duration 
as  the  former  similar  one.  With  the  more  frankly  reactive  types, 
however,  this  becomes  progressively  less  true,  so  that  the  matter  of 
treatment  here  easily  becomes  something  more  than  mere  intelligent 
custodial  care. 

In  the  very  mild  types  of  the  disease  the  patients  have  to  be  care- 
fully safeguarded,  because  their  condition  is  not  appreciated  by  others. 
In  the  excited  phases  alcoholic  and  sexual  excesses  are  especially  apt 
to  occur,  and  it  must  never  be  lost  sight  of  that  during  what  out- 
wardly appear  to  be  the  mildest  depressions  suicide  is  a  possibility. 

In  the  more  pronounced  attacks  the  handling  of  the  patients  calls 
for  the  very  greatest  amount  of  tact.  During  the  excitement  the 
patient's  strength  must  be  guarded,  as  insomnia  is  a  constant  symptom, 
and  food  may  be  taken  in  insufficient  quantities.  Mechanical  restraint 
should,  of  course,  be  avoided  if  possible  and  it  may  be  said  that  it  is 
practically  never  necessary.  Its  application  in  the  high  degrees  of 
excitement  is  often  not  understood  by  the  patient,  and  produces  an 
increase  of  excitement  and  resistance,  and  perhaps  a  state  of  anxious 
apprehension,  and  even  terror.  Chemical  restraint  is  equally  undesir- 
able as  it  tends  to  shut  out  the  real  world  and  thereby  increases  the 
diifficulty  of  adjustment  to  reality.  Hypnotics  may  be  necessary  and 
such  simple  ones  as  veronal  are  the  best — paraldehyd,  trional,  sul- 
phonal,  chloralimid  are  useful,  but  opium  and  its  derivatives  should 
be  avoided  if  possible.  Whereas  the  various  kinds  of  restraint  are 
highly  undesirable,  isolation  may  be  resorted  to  and  is  not  infrequently 
welcomed  by  the  patient.  All  that  may  be  necessary  is  simply  to  put 
the  patient  in  a  room  by  himself  without  locking  the  door.  They  may 
be  very  glad  to  stay  there  and  so  escape  from  sources  of  irritation. 

It  is  in  the  condition  of  excitement  that  the  continuous  bath  is  so 
valuable.  The  patient  is  placed  in  a  tub,  preferably  one  constructed 
especially  for  the  purpose,  being  long  enough  to  permit  the  body  to 
lie  in  it  without  the  limbs  being  flexed.  The  water,  which  should  cover 
the  body  completely,  is  kept  at  a  temperature  of  from  96°  to  98°  F.,  that 
is,  just  above  the  normal  surface  temperature.  It  adds  to  the  comfort 
of  the  patient  if  a  canvass  hammock  can  be  slung  in  the  tub  on  which 
he  can  lay,  and  a  rubber  air  pillow  be  placed  under  his  head.  The  warm 
water  of  the  continuous  bath  is  the  best  sedative  treatment  we  have 
for  this  class  of  patients,  as  it  produces  sedation  without  any  clouding 
of  consciousness  or  other  disagreeable  features.  The  patient  may  be 
left  in  the  tub  for  such  a  period  of  time  as  is  deemed  necessary,  usually 
three  or  four  hours  at  a  time.  On  the  Continent  patients  are  not  in- 
frequently kept  in  the  tub  not  only  for  days,  but  even  months,  sleep- 
ing in  the  tub  and  being  fed  in  it.  On  the  whole  the  patients  enjoy 
this,  the  warm  water  is  soothing,  and  they  are  grateful  for  its  calming 
influence. 

It  is  in  the  excited  phases  that  the  nurses  ingenuity  will  be  taxed 
to  the  utmost,  and  if  she  is  not  tactful  all  sorts  of  artificial  symptoms 


TREATMENT  653 

will  be  created  in  the  way  of  antagonisms  toward  the  nurse,  increased 
irritability,  etc.,  so  that  the  adjustment  of  the  nurse  to  the  patient, 
particularly  in  excited  conditions,  becomes  an  important  practical 
problem. 

In  the  depressed  phase  of  the  disease  the  patient  is  often  best  treated 
in  bed,  particularly  if  the  depression  approaches  the  stuporous  stage. 
Under  these  circumstances  refusal  of  food  is  a  common  symptom  and 
tube  feeding  must  be  resorted  to  at  regular  intervals.  With  the  tube 
feeding  it  is  easy  to  give  such  medicines  as  may  be  required,  either 
hypnotics,  cathartics,  or  anything  else  indicated.  One  must  remember 
that  in  endeavoring  to  probe  the  consciousness  of  depressed  patients 
the  depression  may  be  increased  and  if  suicidal  tendencies  are  present 
this  should  be  borne  in  mind  and  guarded  against. 

If  the  patients  are  stuporous  the  usual  precaution  should  be  taken 
to  see  that  the  position  of  the  body  is  changed  from  time  to  time  and 
that  the  bladder  and  rectum  are  not  permitted  to  become  overloaded. 

The  danger  of  suicide  in  depressed  conditions  is  an  ever-present  one. 
The  only  safe  way  to  deal  with  these  patients  is  to  assume  that  they 
are  all  suicidal.  Probably  one  of  the  reasons  why  more  patients  in  this 
condition  do  not  commit  suicide  is  because  of  the  marked  retardation 
which  makes  it  so  difficult  for  them  to  initiate  any  form  of  activity. 
It  is  the  depressed  patients  who  are  suicidal  who  most  frequently 
require  to  be  sent  to  a  hospital,  although  their  general  condition  may 
not  seem  to  warrant  such  a  move.  The  watching  and  the  caring  for 
a  depressed  patient  with  a  view  to  preventing  them  from  committing 
suicide  is  practically  only  understood  in  institutions  for  the  treatment 
of  mental  disease.  The  general  hospital  nurse,  the  general  practitioner 
and  the  family  rarely  have  any  idea  of  the  degree  of  watchfulness  that 
is  necessary  and  for  this  reason  alone  oftentimes  the  patients  must 
be  sent  to  institutions. 

During  the  period  of  convalescence,  occupation,  outdoor  exercise, 
and  the  like  are  all  in  order.  Care  should  be  taken  not  to  force  the 
patient  too  fast. 

Here  as  elsewhere  in  mental  medicine  an  attempt  should  be  made  to 
analyze  the  mind  sufficiently  at  least  to  understand  the  nature  of  the 
disturbing  factors  that  are  at  work,  and  if  possible  the  way  in  which 
they  have  brought  about  the  psychosis.  This,  of  course,  is  essential  to 
an  intelligent  treatment  of  the  patient.  Such  analysis,  however,  is 
almost  impossible  with  many  patients,  particularly  during  the  attack, 
and  can  only  be  resorted  to  when  the  patient  is  at  least  approaching 
the  normal  condition.  All  the  information  gained,  however,  is  valuable 
as  pointing  the  way  toward  regulating  the  patient's  life  and  in  many 
instances  as  indicating  the  nature  of  the  etiological  factors  and  thereby 
showing  what  must  be  avoided  in  the  future  if  further  attacks  are  to 
be  prevented.  Of  course  much  more  is  to  be  hoped  for  in  the  frank 
reactive  than  in  the  profoundly  constitutional  types. 


654  MANIC-DEPRESSIVE  PSYCHOSIS 

Prophylaxis. — Prophylaxis  resolves  itself  into  two  parts:  first,  the 
prevention  of  the  disease,  and  second,  the  prevention  of  subsequent 
attacks  after  the  disease  has  manifested  itself.  The  prevention  of  the 
disease  is  a  problem  of  eugenics.  We  need  very  much  more  information 
as  to  the  way  in  which  the  manic-depressive  psychosis  conducts  itself 
with  reference  to  the  laws  of  heredity.  It  has  not  as  yet  been  ade- 
quately worked  out  so  that  definite  advice  is  possible. 

As  to  the  prevention  of  subsequent  attacks  the  most  important 
thing  is  to  prevent  if  possible  the  recurrence  of  the  etiological  factors 
that  have  been  found  to  play  a  part  by  the  psychoanalytic  study  of 
the  patient. 


CHAPTER    XVIIL 
THE  PARANOIA  GROUP. 

Perhaps  no  term  in  psychiatry  has  undergone  wider  variations 
of  meaning  than  the  term  paranoia.  In  its  earUest  days,  in  the  Greek 
period,  it  meant  Httle  more  than  just  craziness,  although  perhaps 
it  may  have  been  used  somewhat  more  specifically  in  some  instances, 
and  later  on,  well  into  the  middle  ages,  it  was  still  a  term  that  was 
not  infrequently  used  to  include  the  whole  group  of  the  so-called 
insanities. 

The  term  did  not  come  into  general  use  as  applied  to  a  special  group- 
ing of  mental  symptoms  until  the  early  part  of  the  nineteenth  century, 
when  a  German  psychiatrist,  Heinroth,  made  an  effort  to  classify 
various  mental  diseases  and  gave  paranoia  a  distinct  placement  in 
his  scheme.  The  classifications  of  this  day,  however,  were  extremely 
complex  and  there  was  a  marked  tendency  to  follow  the  dichotomous 
method  with  its  binomial  nomenclature,  which  had  come  into  such 
popular  vogue  with  the  appearance  of  the  work  of  the  great  Swedish 
botanist,  Linnaeus,  in  the  middle  of  the  eighteenth  century.  A  disease 
that  was  classified  under  the  specific  names  of  hallucinatory,  confu- 
sional,  depressed,  or  what  not,  might  change  its  name  and  its  nature 
over  night,  as  it  were.  This  led  to  great  confusion  and  to  the  final 
throwing  out  of  the  whole  scheme  by  the  French,  under  the  leadership 
of  Pinel,  who  reduced  the  classification  to  manias,  melancholias,  and 
dementias.  Esquirol  followed  with  his  monomania,  under  which  the 
paranoias  found  a  place,  and  this  term  has  been  in  use  ever  since, 
largely  by  the  English  school,  and  it  still  finds  application  in  the 
courts.  It  is  based  upon  the  simplistic  conception  that  the  brain  is 
one  organ  and  that  it  has  one  disease,  and  that  disease  is  insanity, 
and  not  only  simplistic  to  this  extent,  but  that  the  disease  may  affect 
any  part  of  the  organ  and  therefore  a  person  may  be  insane  upon  one 
subject,  conceptions  which  are  hardly  worthy  of  a  school  boy,  but 
yet  are  still  held  in  some  quarters  today. 

From  this  time  on  the  general  concept  of  paranoia  became  some- 
what more  definite  and  it  tended  more  and  more  to  concentrate  and 
crystallize  itself  about  a  condition  which  presented  essentially  delu- 
sions, more  or  less  clearly  formed  and  of  a  persecutory  type  generally 
associated  with  hallucinations,  especially  auditory.  Even  this  con- 
cept, however,  included  such  a  mass  of  material  of  such  dissimilar 
types  that  it  never  became  very  well  defined.  The  paranoia  contro- 
versy during  this  period,  namely  from  the  middle  to  the  end  of  the 


656  THE  PARANOIA   GROUP 

nineteenth  century,  is  largely  taken  up  with  a  discussion  upon  the 
basis  of  the  old  facultj'^  psychology,  and  the  division  of  the  mind  into 
three  parts,  the  intellect,  the  emotions,  and  the  will.  There  had 
already  been  a  tendency  to  consider  paranoia  as  a  primary  intellec- 
tual disorder.  The  discussion  took  up  the  question  as  to  whether 
the  emotions  were  involved  primarily;  secondarily,  or  at  all.  This 
whole  bootless  procedure,  based  upon  the  faculty  psychology,  of 
course  came  to  naught,  simply  because  the  mind  is  not  split  up  into 
mutually  exclusive  compartments.  Another  one  of  the  concepts  upon 
which  such  discussions  were  based,  and  which  was  assumed  in  the  dis- 
cussion, was  that  there  were  such  things  as  mental  disease  entities 
which  had  as  much  individuality  and  definiteness  in  the  conceptions 
of  the  psychiatrist  as  tumors  had  in  the  minds  of  the  pathologist. 

Without  going  into  a  description  of  the  different  ideas  of  paranoia 
which  have  been  extant,  and  which  in  their  later  development  will 
be  described  in  the  body  of  the  chapter,  it  need  only  be  added  that 
the  general  result  of  all  this  discussion  is  first  that  the  brain  is  not  a 
single  organ.  It  is  a  great  number  of  organs  crowded  into  a  very  close 
space  and  that  the  functions  of  its  different  parts  need  be  no  more 
closely  related  to  one  another  than  the  functions  of  the  adrenal  glands 
and  the  hypophysis.  The  cortex  alone  consists  of  at  least  fifty  his- 
tologically differentiated  organs,  while  the  thalamus  is  composed  of  at 
least  nine  ganglia.  The  red  nucleus  is  an  organ  by  itself,  as  are  the 
different  portions  of  the  lenticular  nucleus,  and  the  separate  ganglia 
innervating  the  ocular  muscles.  In  some  way  or  other  there  issues 
from  this  complex  of  organs,  or  more  properly  is  associated  with  it, 
the  phenomena  of  mind.  Mind  is  not  a  single  thing  any  more  than 
is  the  brain.  It  is  not  only  as  complex  as  the  organ  which  subserves 
its  function,  but  infinitely  more  complex  than  this  organ  as  we  know 
it  today.  The  mind  cannot  be  conceived  as  divided  into  compart- 
ments like  the  will,  the  intellect,  and  the  feelings,  each  presided  over 
by  a  mythological  demon,  so  to  speak,  but  must  be  conceived  of  as  a 
complex  of  adaptive  mechanisms  interrelated  with  one  another  in  the 
most  intricate  manner,  so  that  the  mind  must  be  conceived  of  as 
capable  of  having  not  only  one,  but  many  kinds  of  disorders,  which 
disorders  are  not  entities  in  the  sense  of  foreign  bodies  or  diseases 
which  enter  from  outside,  but  are  inefficient  ways  of  functioning, 
special  combinations  of  mechanisms,  and  so  we  have  not  so  much 
mental  diseases  after  all  as  types  of  mental  reaction.  The  disease  is 
not,  therefore,  something  which  comes  from  without,  but  it  is  a  func- 
tion of  the  interrelation  between  the  individual  and  his  environment, 
and  only  in  proportion  as  this  interrelation  is  inefficient  may  it  be 
conceived  of  as  disease,  and  only  in  accordance  with  the  type  of 
mechanism  which  is  utilized,  the  special  trend  of  reaction,  can  we  speak 
of  a  disease  in  any  specific  sense  at  all.  This  is  quite  parallel  with 
the  concepts  on  the  physical  side  and  is  important  to  bear  in  mind  if 
we  are  not  to  be  enslaved  by  a  limiting  terminology. 


DESCRIPTION  657 

The  most  recent  advances,  therefore,  in  the  concept  of  paranoia 
are,  a  getting  away  from  the  consideration  of  it  as  a  disease  entity, 
or  as  involving  a  special  faculty  of  the  mind,  or  as  a  merely  mono- 
symptomatic  classification,  and  a  coming  to  consider  it  as  a  type  of 
reaction  which  manifests  itself  in  certain  individuals,  probably  as  the 
result  of  certain  specific  types  of  noxa.  The  descriptive  attitude 
toward  the  problem  is  being  replaced  by  the  interpretative.  This  is 
significant,  as  paranoia  has  long  been  the  stronghold  for  descriptive 
psychiatry  and  it  has  been  the  last  to  yield  to  anything  like  an  inter- 
pretative approach. 

Description. — The  general  concept  of  paranoia  which  has  been  preva- 
lent for  many  years  is  that  of  a  psychosis  presenting  delusions  of  per- 
secution of  a  pretty  clearly  defined  type,  well  supported  and  defended 
by  the  patient,  in  other  words  systematized.  These  delusions  generally 
involve  a  more  or  less  circumscribed  portion  of  the  mentality,  although 
they  tend  to  spread  out  slowly  and  involve  more  and  more.  With 
this  state  of  mind  there  is  no  marked  tendency  toward  deterioration, 
the  disease  having  essentially  a  chronic  course  and  remaining  unchanged 
for  years.  Associated  with  the  delusions  and  harmonized  with  them 
in  content  are  frequently  auditory  hallucinations — ^voices. 

This  is  the  general  concept  of  the  disease  which  has  received  various 
modifications  and  descriptive  clothings  by  different  authors.  For 
many  years  the  delire  chronique  a  evolution  sysUmatique  of  Magnan 
in  France  and  the  paranoia  of  Krafft-Ebing  in  Germany  have  been 
the  paradigma  under  which  the  various  forms  have  been  arranged. 
The  delire  chronique  of  Magnan  was  a  disease  which  progressed 
regularly  through  four  stages :  first,  a  hypochondriacal  stage,  or  stage 
of  subjective  analysis;  second,  a  stage  of  persecution;  third,  a  stage  of 
transformation  of  the  personality;  and  sometimes,  fourth,  a  stage  of 
dementia. 

In  the  first  or  the  hypochondriacal  stage,  or  stage  of  subiective 
analysis,  the  patient  is  self-centered  and  depressed  and  has  ideas  of 
reference.  He  also  complains  of  many  physical  symptoms,  such  as 
dizziness,  weakness,  headaches,  etc.  Everything  that  happens  about 
him  tends  to  be  referred  to  himself,  so  that  he  is  in  a  constant  state 
of  morbid  introspection  about  things  which  he  does  not  understand. 
In  the  second  stage  the  explanation  of  all  these  things  finds  itself  in 
the  delusions  of  persecution.  The  reasons  why  people  have  slighted 
him,  why  they  have  said  disagreeable  things  about  him,  why  they 
talk  about  him  and  spread  rumors  about  him  is  all  understandable 
because  of  the  conspiracy  which  there  is  against  him.  These  delu- 
sions are  reenforced  often  by  hallucinations  of  hearing,  and  he  hears 
actual  evidences  of  all  of  the  things  which  are  being  done  to  annoy, 
to  persecute,  or  to  destroy  him.  Explanatory  delusions  follow  which 
give  the  reasons  to  the  patient  why  he  is  thus  persecuted,  and  usually 
attribute  the  persecution  to  some  special  person  or  group  of  persons, 
or  some  society  or  institution.  In  this  persecutory  period  the  patient 
42 


658  THE  PARANOIA   GROUP 

may  be  very  dangerous.  He  may  react  upon  his  persecutors  if  he 
knows  who  they  are,  that  is,  if  he  has  defined  them  in  his  own  mind. 
On  the  other  hand,  he  may  develop  elaborate  defenses  against  them  or 
may  endeavor  to  flee  from  them  entirely.  Later  on,  as  a  result  of  the 
elaboration  of  the  whole  system  with  the  effort  at  finding  further 
explanations  for  them  all,  there  are  developed  the  ideas  of  self-impor- 
tance. The  patient  concludes  that  if  so  many  people  are  leagued 
against  him  that  he  must  indeed  be  an  important  personage.  When 
this  occurs  we  are  in  the  face  of  the  third  phase  of  the  disorder,  namely, 
the  transformation  of  the  personality.  By  this  time  the  delusional 
system  has  become  extremely  complex,  with  interminable  ramifica- 
tions in  all  directions  involving  all  sorts  of  occurrences,  all  manner  of 
people,  and  reaching  backward  into  the  past  and  finding  delusional 
explanations  of  events  even  in  the  patient's  childhood.  These  explana- 
tions often  refer  to  facts  which  antedate  the  period  of  the  psychosis, 
and  are  therefore  known  as  retrospective  falsifications.  Following 
the  transformation  of  the  personality  there  may  occur  a  certain  amount 
of  mental  enfeeblement. 

This,  in  a  few  words,  is  Magnan's  delire  chronique,  a  disease  which 
is  extremely  rare  if  this  gradual  evolution  of  the  several  stages  described 
by  him  is  looked  for ;  that  it  does  occur  from  time  to  time  cannot 
be  disputed.  In  all  probability  a  number  of  cases  of  paranoid 
dementia  precox  were  included  in  this  group,  while  today  the  group 
has  been  recognized  in  a  much  restricted  sense  by  Kraepelin^  in  his 
paraphrenias. 

Krafft-Ebing,  in  Germany,  developed  a  picture  of  the  disease  which 
for  many  years  had  much  vogue  in  this  country,  and  found  particular 
favor,  largely,  probably  because  of  its  easy  applicability.  In  the 
first  place  he  divided  all  paranoias  into  two  great  groups,  the  original 
and  the  acquired.  The  original  paranoias,  after  Sander,  were  those 
which  presented  their  psychosis  as  the  natural  unfolding  of  an  abnor- 
mal character,  while  the  acquired  or  late  'paranoias  were  the  paranoias 
which  developed  in  people  who  up  to  a  certain  point  had  appeared 
normal.  A  further  subdivision  of  these  groups  was  into  paranoia 
halhicinatoria,  in  which  hallucinations  played  a  prominent  part,  and 
paranoia  combinatoria,  in  which  they  were  absent  or  of  minor  impor- 
tance. Following  these  great  groups  there  were  secondary  groups 
which  stood  upon  a  purely  symptomatic  basis,  and  so  he  described 
persecutory  paranoia,  expansive  paranoia,  querulous  or  litigious  para- 
noia, and  inventive,  reformatory,  religious,  and  erotic  types. 

More  recently  there  has  been  a  tendency  to  gradually  restrict  the 
paranoia  group  to  narrower  and  narrower  limits.  One  of  the  most 
recent  attempts  at  more  clear  definition  is  that  of  the  French  authors, 
Serieux  and  Capgras,^  who  have  described  two  types  of  psychosis 

1  Psychiatrie,  8th  Auflage. 

^  Les  folies  i  aisonnantes,  Paris,  1909;  and  Diagnostic  du  delire  d'interpretation, 
Revue   de   Psychiatrie,   January,    1908. 


DESCRIPTION  659 

to  which  alone  they  think  the  term  paranoia  apphcable,  namely,  the 
delirium  of  interpretation  and  the  delirium  of  revindication.  In  the 
delirium  of  interpretation  the  patient  has  ideas  of  reference,  and  because 
of  his  lack  of  critique  and  egocentricity  comes  to  all  sorts  of  false 
interpretations  of  what  is  going  on  about  him.  These  delusional  inter- 
pretations become  systematized  and  reach  more  or  less  coherency 
without  any  special  dependence  upon  disorders  of  the  sensorium. 
There  is  no  deterioration,  and  lucidity  is  maintained  throughout  the 
evolution  of  the  psychosis.  Unlike  certain  other  paranoid  conditions 
the  false  interpretations  have  their  origin  in  actual  facts. 

In  the  deliriujn  of  revindication  a  chronic  systematized  psychosis 
which  takes  its  origin  in  a  fixed  idea  appears.  It  is  a  monoideism,  and 
its  various  ramifications,  like  the  other  form  of  paranoia,  do  not  tend 
toward  dementia.  They  describe  two  varieties  of  this  psychosis,  the 
egocentric  type,  the  subject  of  which  are  usually  persecutors  making 
claims  for  wrongs  suffered  that  may  or  may  not  have  some  founda- 
tion in  fact.  Then  there  is  the  altruistic  type,  characterized  by  abstrac- 
tions and  impersonal  theories.  To  this  group  belong  the  inven- 
tors, the  reformers,  and  the  prophets,  becoming,  however,  in  their 
endeavor  to  realize  their  ideals,  ofttimes  dangerous  fanatics,  mystics, 
anarchists,  regicides. 

In  Germany  Kraepelin  limited  the  paranoia  concept  perhaps  more 
than  any  one  else.  He  confined  the  term  to  a  very  circumscribed  and 
very  small  group.  His  conception  of  the  disease  is  a  chronic  incur- 
able psychosis  of  insidious  origin  developing  slowly  by  the  gradual 
systematizing  of  endogenous  delusions.  This  system  of  delusions  is 
enduring  and  unshakable  and  exists  along  with  the  retention  of  the 
logical  and  orderly  process  of  thinking.  There  is  no  marked  tendency 
to  mental  deterioration,  and  hallucinations  play  no  essential  part  in 
the  picture. 

Kraepelin  has  recently,  in  the  eighth  edition  of  his  work,  still  more 
clearly  defined  his  paranoid  group  by  describing  a  group,  paraphrenia, 
which  contains  certain  paranoid  types  that  closely  resemble  his  para- 
noia, but  which  provisionally  he  includes  in  this  group  for  purposes 
of  greater  definition.  This  group  of  paraphrenia  is  divided  into  four 
sub-groups,  as  follows : 

Paraphrenia  systematica,  which  is  for  the  most  part  Magnan's 
delire  chronique,  with  the  exception  that  the  well-defined  precox 
types  with  marked  deterioration  are  excluded. 

Paraphrenia  Expansii}a. — This  form  aftects  only  women  and  is 
marked  by  the  development  of  ideas  of  grandeur  with  mild  excitement 
and  exaltation.    No  dementia  follows. 

Paraphrenia  Confahulans. — Here  the  delusions  both  of  persecution 
and  grandeur  are  specially  marked  by  their  foundation  upon  and 
reference  to  memory  falsifications,  as  the  name  indicates. 

Paraphrenia  phantastica  is  the  term  applied  to  cases  with  a  certain 
amount  of  exaltation,  with  the  recounting  of  remarkable  adventures 


660  THE  PARANOIA   GROUP 

and  incoherent  changing  delusions.  This  condition  is  characteristically 
accompanied  by  hallucinations  of  hearing.  This  group  includes  the 
cases  previously  described  by  him  under  the  term  dementia  paranoides. 

Interpretation. — The  rather  simplistic  attitude  which  dominated 
shortly  after  the  term  paranoia  came  into  general  use  and  which  saw 
in  every  combination  of  fairly  well-defined  and  fixed  persecutory 
ideas,  especially  those  supported  by  hallucinations  of  hearing,  the 
disease  paranoia  had  to  give  way  in  a  very  few  years  to  a  broader, 
if  somewhat  less  well-defined,  attitude.  It  soon  became  evident  that 
a  fairly  systematized  and  fixed  delusional  system  of  persecutory  charac- 
ter might  occur  as  the  expression  of  a  psychosis  from  which  recovery 
took  place.  And  so  the  element  of  the  concept  of  paranoia  which 
considered  it  as  essentially  chronic,  progressive,  and  incurable  had 
to  be  readjusted.  These  so-called  acute  paranoias  have  been  recently 
studied  quite  extensively  by  Friedmann,^  and  their  origin  traced  to 
actual  situations  in  the  patient's  life,  so  that  the  delusions  appear  as 
logical  outgrowths  of  experience,  and  have  as  a  consequence  fallen 
into  the  group  of  the  psychogenic  psychoses. 

Not  only  was  the  idea  of  chronicity  associated  with  paranoia  seriously 
shaken,  but  from  other  sources  the  idea  of  the  specificity  of  the  per- 
secutory delusion  also  had  to  give  way,  for  it  was  soon  found  that 
ideas  of  persecution  of  paranoiac  character  were  not  at  all  infrequent 
in  connection  with  other  psychoses.  This  was  particularly  evident  in 
the  psychoses  of  chronic  alcoholism.  It  soon  developed  that  there 
was  a  special  form  of  dementia  precox  presenting  paranoid  ideas, 
while  later  studies  showed  luetic  forms  with  paranoid  symptomatology, 
presenile  forms,  paranoid  states  of  mind  of  the  deaf,  and  others  who 
are  isolated  from  close  contact  with  the  world,  to  say  nothing  of  the 
recent  paraphrenia  group  of  Kraepelin  and  many  other  less  well-defined 
conditions,  which  have  included  more  recently  not  only  the  manic- 
depressive  psychosis,  but  certain  of  the  milder  cyclothymic  manifes- 
tations of  this  disorder. 

From  these  considerations  it  appears  that  here,  as  elsewhere  in  the 
field  of  psychiatry,  that  the  important  thing  to  consider  is  not  so  much 
the  special  content  of  the  particular  psychosis  in  a  given  individual 
as  the  mechanisms  which  are  involved,  for  here  we  see  a  similar  con- 
tent in  all  sorts  of  mental  disorders,  some  acute,  some  chronic,  and  are 
therefore  forced  to  look  beneath  and  see  whether  it  is  not  possible  to 
understand  these  manifestations  by  attributing  them  to  a  common 
mechanism. 

The  studies  of  Friedmann,  already  mentioned,  went  a  long  way 
toward  showing  the  dependence  of  paranoid  trends  upon  actual  situa- 
tions in  the  patient's  life,  and  demonstrated  how  paranoid  delusions 
in  given  cases  might  grow  as  a  result  from  these  situations.    In  other 

1  Contributions  to  the  Study  of  Paranoia  in  Studies  in  Paranoia,  Nervous  and  Mental 
Disease  Monograph  Series,  No.  2. 


INTERPRETATION  661 

words,  they  are  of  psychogenic  origin  and  are  perfectly  understand- 
able when  all  of  the  circumstances  have  been  uncovered. 

Among  others,  Gierlich^  has  shown  that  paranoid  ideas  often 
accompany  fluctuations  of  affect  which  could  only  be  considered  as 
manifestations  of  a  manic-depressive  psychosis  and  that  many  of  the 
paranoid  conditions  which  were  associated  with  only  slight  affect 
manifestations  which  ■  belonged  to  the  manic-depressive  psychosis 
might  easily  be  overlooked  as  coming  under  that  group  and  be  mis- 
taken for  true  paranoia.  In  this  way  he  accounted  for  a  very  large 
number  at  least  of  the  so-called  acute  paranoias,  as  these  patients  of 
course  got  well  from  the  attacks  as  the  manic-depressive  casesusually  do. 

More  recently  Specht'^  has  at  great  length  endeavored  to  demonstrate 
that  the  underlying  condition  in  paranoia  was  an  affect  of  suspicious- 
ness and  therefore  he  brought  the  paranoid  group  into  close  alliance 
with  the  great  affect  group  of  the  psychoses,  namely,  the  manic-depres- 
sive group.  This  whole  discussion  has  broadened  out  in  all  directions 
and  has  become  very  complex  and  extremely  involved,  and  therefore 
it  is  not  a  proper  subject  for  further  elaboration  in  a  text-book.  It 
might  be  added,  however,  that  Blueler,^  who  has  made  a  most  incisive 
study  of  the  psychology  of  paranoia,  denies  absolutely  that  suspicious- 
ness is  an  affect  at  all,  and  therefore  departs  radically  from  Specht's 
position.  He  believes  suspiciousness  is  a  state  of  mind  based  entirely 
upon  perceptions  and  the  resulting  conclusions,  and  is  therefore  of 
purely  intellectual  origin,  but  that  it  is  accompanied  by  affect,  as  are 
all  mental  states.  Here,  again,  the  fallaciousness  of  the  old  faculty 
psychology  that  would  separate  the  mind  into  different  parts,  such  as 
the  intellect  and  the  emotions,  should  be  emphasized.  The  two 
invariably  occur  together,  and  suspiciousness  of  course,  therefore  is 
accompanied  by  its  affect. 

Blueler  is  of  the  opinion  that  paranoia  takes  its  origin  in  certain 
constellations  of  ideas  or  complexes  and  the  dominant  affect  with 
which  they  are  loaded,  that  these  complexes  are  precisely  of  the  same 
nature  as  are  found  in  normal  individuals,  and  that  the  abnormal 
element  which  leads  to  the  elaboration  of  a  psychosis  is  the  fixation 
upon  this  complex,  the  inability  to  get  away  from  it,  or  as  might  be 
said,  the  inability  to  reach  an  efficient  adjustment  to  it. 

One  here  sees  what  is  everywhere  apparent  in  dealing  with  mental 
disorders,  that  the  delusion  is  not  the  disease,  the  delusion  is  only 
one  expression  of  the  disease.  The  mechanism  involved  has  to  deal 
with  a  certain  content;  this  content  is  delusional,  but  is  therefore 
only  the  outward  expression  of  the  disorder  beneath.  Therefore  the 
delusion  really  expresses  an  effort  upon  the  part  of  the  individual  to 

1  Periodic  Paranoia  and  the  Origin  of  Paranoid  Delusions  in  Studies  in  Paranoia, 
Nervous  and  Mental  Disease  Monograph  Series,  No.  2. 

2  Ueber  den  pathologischen  affect  in  der  chronischen  Paranoia.  Cited  by  Bleuler, 
in  Affectivity,  Suggestibility,  Paranoia,  New  York  State  Hospital  Bulletin,  vol.  iv. 

3  Affectivity,  Suggestibility,  Paranoia,  New  York  State  Hospital  Bulletin,  vol.  iv,' 
February  15,  1912. 


662  THE  PARANOIA   GROUP 

reach  an  efficient  adjustment.  The  constellation  of  ideas  with  its 
dominant,  painful  affect  has  been  one  to  which  the  patient  could  not 
effectively  relate  himself,  and  therefore  the  next  best  thing  had  to 
be  done,  and  this  next  best  thing  was  the  formation  of  certain  delu- 
sions which  rendered  the  existence  of  the  painfully  affected  complex 
more  endurable.  The  delusion,  therefore,  speaking  in  physical  terms, 
is  more  comparable  to  scar  tissue  than  to  disease  tissue.  It  represents 
the  location  of  the  wound  and  the  result  of  the  reparative  process. 
To  make  the  matter  more  clear,  an  individual  who  is  ambitious,  and 
yet  who  lacks  ability,  may  develop  the  delusion  that  his  lack  of  success 
is  due,  not  to  his  lack  of  ability,  which  he  persistently  refuses  to  see, 
but  to  the  interference  of  enemies  who  are  jealous  of  him  and  who 
persecute  him  and  try  to  belittle  him  in  the  eyes  of  his  superiors.  In 
this  way  an  inacceptable  fact — his  inefficiency  to  which  he  cannot 
make  adequate  adjustment — is  so  distorted  that  it  would  appear  that 
the  results  of  this  inefficiency  emanate  not  from  within,  but  from 
interferences  from  without.  And  so  the  patient  creates  a  situation 
in  which  he,  so  to  speak,  finds  himself  able  to  get  along,  for  as  painful 
as  a  system  of  persecution  of  the  sort  which  he  creates  for  himself 
may  be  to  him,  it  is  less  so  than  a  realization  of  his  own  inherent 
defects.  It  will  be  seen,  therefore,  what  is  meant  when  it  is  said  that 
the  delusion,  speaking  in  physical  terms,  represents  scar  tissue,  and 
it  will  be  seen  also  how  a  destruction  of  the  delusion  could  in  no  way 
cure  the  disease.  If  the  delusion  could  actually  be  destroyed  the 
patient  would  be  in  much  the  same  position  as  a  patient  who  had  a 
scar  cut  out;  another  delusion  would  have  to  take  its  place,  because 
the  patient  would  be  thrown  back  upon  the  same  inacceptable 
situation  to  which  he  would  find  himself  again  incapable  of  making 
efficient  adaptation. 

Another  mechanism  which  is  revealed  in  this  illustration  is  of  great 
importance  as  being  'characteristic  of  the  paranoid  reaction  type, 
namely,  the  mechanism  of  projection,  whereby  the  individual  projects, 
as  it  were,  upon  the  outside  world  his  own  mental  difficulties  which 
return  to  him  in  this  instance  in  the  form  of  persecution.  This 
mechanism  of  projection  is  a  very  common  one  and  appears  to  be 
fundamental  in  paranoid  trends.  It  is  at  the  basis  also  of  the  ideas 
of  grandeur.  Here  the  patient  projects,  not  his  difficulties,  but  his 
ambitions,  and  his  hopes  come  back  to  him  from  the  outer  world 
realized. 

The  most  elaborate  attempt  at  interpretation  of  the  paranoia  syn- 
drome was  made  recently  by  Freud^  in  his  analysis  of  the  Schreber 
case.  Herein  Freud  voiced  the  view  that  paranoia  was  dependent 
upon  a  homosexual  fixation  in  the  psychosexual  development  of  the 

'  Psychoanalytische  Bemerkungen  ilber  einen  autobiographischen  beschreibenen  Fall 
von  Paranoia  (Dementia  paranoides),  Jahrbuch  fur  psychoanalytische  und  psycho- 
pathologische  Forschungen,  Bd.  iii,  1911.  A  very  excellent  and  full  account  in  English 
of  Freud's  analysis  of  this  case  can  be  found  in  the  Psychoanalytic  Review,  vol.  i.  No.  1. 


DIAGNOSIS  663 

individual.  To  make  this  statement  somewhat  clearer  it  should  be 
recalled  that  the  individual  in  his  psychosexual  development  is  first 
auto-erotic,  that  is,  interested  only  in  his  own  body,  that  his  next 
interest  is  in  those  immediately  about  him,  the  members  of  his  own 
family,  and  particularly  those  of  his  own  sex,  in  other  words,  those 
who  have  bodies  most  like  his  own,  and  that  these  stages  have  to  be 
passed  through  before  the  normal  end-result  in  a  heterosexual  object 
love  is  attained.  The  unconscious  homosexual  interest  in  the  members 
of  his  own  family  is  designated  as  narcissism,  and  the  paranoiac  mechan- 
ism is  dependent  upon  a  fixation  and  development  at  this  period.  In 
the  normal  development  of  the  individual  the  unconscious  homosexual 
tendencies  are  not  entirely  eliminated  by  any  means,  but  the  homo- 
sexual libido  is  sublimated,  that  is,  its  energies  are  utilized  in  other 
channels,  more  particularly  it  is  utilized  in  all  those  forms  of  associa- 
tion with  the  same  sex  that  one  sees  in  friendships,  social  organizations, 
clubs,  games,  and  in  the  higher  social  activities.  But  with  a  fixation- 
point  at  the  narcissistic  period  of  psychosexual  development  the  patient 
is  constantly  in  danger.  Any  serious  conflict  is  liable  to  cause  a 
regression  of  the  sublimation  to  the  point  of  fixation,  and  this  is 
considered  by  Freud  to  be  the  mechanism  at  the  basis  of  paranoia. 
"Persons  who  cannot  rise  completely  out  of  the  stage  of  narcissism 
and  are  thus  prematurely  fixed  or  arrested  in  the  evolution  of  their 
dispositions,  are  exposed  to  the  danger  that  a  flood  of  libido  which 
finds  no  outlet,  sexualizes  their  social  tendencies  and  reverts  the 
sublimations  achieved  in  the  course  of  development."  The  libido  of 
the  paranoiac  is  then  projected  upon  those  about  him. 

The  whole  process  is  briefly  and  ingeniously  set  forth  by  Freud  by 
means  of  ringing  the  changes — supposing  the  paranoiac  to  be  a  male — 
upon  the  basal  sentence  "I  love  him,"  thus: 

Delusions  of  persecution  contradict  the  verb.  "I  love  him"  is 
resented  by  the  individual  who  reacts  to  the  feeling  by  "I  do  not 
love — I  rather  hate  him."  Then  this  feeling  of  hate  is  projected  with 
the  result  "he  hates  (persecutes)  me,  which  justifies  my  hating  him." 
As  a  result,  this  feeling,  appearing  to  come  from  an  outer  perception 
becomes  "  I  really  do  not  love  him — I  hate  him — because  he  persecutes 
me." 

Erotomania  contradicts  the  object.  "I  do  not  love  him — I  love 
her,"  then  "I  notice  that  she  loves  me,"  then  finally,  "I  do  not 
love  him — I  love  her — because  she  loves  me." 

Delusions  of  jealousy  contradict  the  subject.  "  Not,  I  love  the  man — 
she  loves  him." 

Delusions  of  grandeur  result  from  a  total  contradiction,  a  rejec- 
tion of  the  whole  sentence.  "I  do  not  love  at  all,"  and  hence,  "I 
love  nobody."  As  the  libido  must  be  accounted  for  this  is  equivalent 
to  "I  love  only  myself." 

Diagnosis. — Attention  has  already  been  called  in  the  body  of  this 
chapter  to  the  different  conditions  which  have  to  be  borne  in  mind 


664  THE  PARANOIA   GROUP 

in  making  a  diagnosis.  There  are  many  paranoid  states,  and  wherever 
the  paranoid  mechanism  is  present  then  it  is  proper  to  speak  of  a  para- 
noid state.  These  paranoid  states  are  found  in  many  of  the  psychoses. 
They  may  be  more  or  less  permanently  associated  with  the  special 
attack,  as  in  the  manic-depressive  psychosis,  or  as  in  alcoholic  hallu- 
cinosis, or  there  may  be  transient  episodes,  as  in  general  paresis.  It 
will  be  seen  therefore  that  there  are  many  and  various  types  of  symp- 
tomatic paranoid  states  and  that  perhaps  the  main  consideration  in 
the  matter  of  diagnosis  is  that  a  condition  which  is  symptomatic  and 
transitory  should  not  be  mistaken  for  a  chronic,  progressive,  and 
probably  irrecoverable  psychosis.  This  differentiation  cannot  always 
be  made  on  the  basis  of  a  cross-section,  but  the  patient  must  be  studied 
carefully  over  a  considerable  period  of  time,  and  a  reasonably  full 
history  antedating  the  period  at  which  he  came  under  observation 
must  also  be  had  in  order  to  see  what  the  general  progress  indicates. 

Treatment. — For  a  considerable  time  past  the  general  attitude  toward 
the  group  of  cases  included  under  the  designation  of  paranoia  has 
been  that  they  were  incurable.  The  outlook  has  been  an  extremely 
dark  and  pessimistic  one,  and  correspondingly  therapeutic  efforts 
have  been  paralyzed  at  their  very  inception.  A  somewhat  changed 
attitude  toward  the  whole  group  was  the  natural  result  of  the  develop- 
ment of  a  concept  of  paranoia  which  was  more  circumscribed  and 
applicable  to  a  more  limited  number  of  patients,  and  when  corre- 
spondingly it  was  learned  that  there  were  many  paranoid  states 
associated  with  essentially  recoverable  psychoses;  in  other  words,  as 
the  paranoia  concept  has  become  more  and  more  contracted  it  has 
been  realized  that  a  great  many  of  the  paranoid  conditions,  which 
formerly  were  grouped  under  the  head  of  paranoia,  really  belonged  to 
recoverable  transient  conditions,  and  therefore  the  outlook  for  them 
was  good.  On  the  other  hand,  as  the  paranoia  concept  has  contracted 
it  cannot  be  said  that  there  has  been  any  increase,  at  least  until  very 
recently,  in  the  hopefulness  for  this  limited  group  of  cases. 

It  could  hardly  be  expected  that  at  a  time  when  paranoia  was 
considered  to  be  an  absolutely  hopeless  and  irrecoverable  psychosis, 
chronic  and  progressive  in  its  very  nature,  that  therapeutic  results 
would  offer  much,  or  that  there  would  be  found  many  who  would 
even  give  any  material  effort  in  this  direction,  but  here  and  there 
scattered  through  the  literature  are  reports  of  cases  of  paranoid  type 
which  seemed  to  have  been  influenced  by  this  or  that  form  of  thera- 
peutic procedure,  and  more  recently,  since  the  doors  have  been  opened 
and  one  has  been  able  to  enter  more  intimately  into  a  knowledge  of 
the  mechanisms  that  are  involved  in  the  development  of  the  psychoses 
and  when  it  has  been  seen  that  these  mechanisms  at  least  are  quite 
the  same  in  mental  disease  as  they  are  in  health  even,  and  that  the 
mechanisms  of  chronic  psychoses  are  quite  the  same  as  those  found  in 
recoverable  conditions  and  also  in  health,  and  that  the  abnormal 
feature  was  not  so  much  the  mechanism  as  the  fixation  of  the  individ- 


TREATMENT  665 

ual  at  certain  periods  of  development  or  with  reference  to  certain 
constellations  of  ideas,  it  became  at  once  an  open  question  whether 
these  conditions  might  not  be  susceptible  of  the  same  sort  of  modifi- 
cation as  they  are  in  less  serious  conditions.  And  so  within  the  past 
few  years  there  has  been  an  awakening  of  interest  in  these  chronic 
psychoses  and  efforts  are  being  made  here  and  there  to  penetrate  their 
mysteries  and  to  modify  their  course,  with  the  result  that  already  a 
number  of  cases  of  paranoia  have  been  reported  as  having  had  a  favor- 
able outcome. 

The  general  principle  of  treatment,  at  the  psychological  level, 
resolves  itself  into  as  complete  as  possible  an  unraveling  of  the  tangled 
skein  of  the  patient's  mental  life,  an  uncovering  of  the  activating  cir- 
cumstances in  his  career  which  have  been  the  etiological  factors  in 
the  development  of  the  psychosis,  and  by  so  doing  modifying  his 
mental  trends  by  a  progressive  process  of  readjustments  and  reeduca- 
tion. This  is  the  work  of  one  skilled  in  the  analysis  of  psychological 
situations  and  is  of  quite  the  same  nature  as  the  psychoanalytic  treat- 
ment of  the  neuroses. 

It  seems  certain,  from  the  results  of  dealing  with  these  paranoid 
conditions,  that  aside  from  any  definite  ability  to  modify  the  course 
of  the  psychosis  or  to  produce  a  definite  curative  result,  that  the 
psychoanalytic  method  of  attack  may,  not  infrequently  at  least,  lead 
to  a  certain  amount  of  transfer,  that  the  physician  may  come  to  be 
highly  respected  and  affectionately  regarded  by  the  patient  to  such 
an  extent  at  least  that  he  may  very  largely  control  the  patient's 
activities;  This  has  been  known  to  happen  under  rather  extraordinary 
circumstances,  showing  a  very  high  degree  of  personal  influence  by 
the  physician  over  the  patient,  despite  the  fact  of  well-marked  and 
fixed  delusional  beliefs. 

In  dealing  with  paranoiacs  it  must  always  be  remembered  that  one 
is  dealing  with  a  class  of  patients  who  are  potentially  dangerous, 
that  to  this  group  belong  perhaps  the  most  dangerous  of  the  so-called 
insane,  and  aside  from  matters  of  psychoanalysis,  or  in  fact  of  any 
questions  of  therapeutic  endeavor,  it  must  be  realized  that  where  it 
is  evident  that  the  welfare  of  the  individual  and  the  welfare  of  society 
cross,  the  welfare  of  the  individual  must  give  way  in  favor  of  that  of 
society.  If  the  paranoiac  is  actually  dangerous  it  is  necessary  to  interne 
him  in  some  institution  where  he  will  get  proper  care. 

The  question  as  to  whether  a  given  paranoiac  is  dangerous  or  not, 
in  the  absence  of  any  overt  acts,  is  often  an  extremely  difficult  one 
to  decide.  In  any  case  it  is  a  question  to  be  decided  by  a  study  of 
the  individual  case  and  it  always  includes  a  consideration  of  many 
factors.  Among  these  factors  the  following  may  be  mentioned:  It 
is  important  to  find  out  how  completely  the  mentality  of  the  patient 
is  permeated  by  the  delusional  system,  in  other  words,  how  much  or 
how  little  freedom  he  has  from  delusional  control,  whether  all  of  his 
mental  forces,  so  to  speak,  go  to  reenforce  the  delusion,  or  whether. 


666  THE  PARANOIA   GROUP 

on  the  other  hand,  he  is  left  reasonably  free  for  a  considerable  portion 
of  the  time,  in  contact  with  reality,  rather  than  plunged  into  the  depths 
of  his  unreal  world.  It  is  important,  too,  to  note  how  clearly  defined 
may  be  his  belief  in  the  activity  of  any  specific  individual  in  his 
delusional  system,  whether  he  believes  some  person  who  is  living, 
perhaps  nearby,  someone  whom  he  can  easily  come  in  contact  with, 
is  responsible,  at  the  bottom  of  his  persecutions.  It  is  important  to 
see  whether  the  patient,  in  the  consideration  of  his  delusional  ideas, 
is  at  all  subject  to  the  reality  motive,  whether  he  has  any  critique  left, 
or  whether  his  belief  is  shakable  in  any  degree  by  others,  whether  he 
can  be  influenced  materially  by  his  physician  when  it  comes  to  the 
question  of  his  delusional  beliefs,  or  whether  they  dominate  the  situa- 
tion absolutely.  It  is  important  to  judge  the  general  attitude  and  mood 
of  the  individual,  whether  he  is  entirely  shut  out  from  any  considera- 
tion of  others,  of  the  world  at  large,  whether  he  considers  himself 
quite  a  law  unto  himself,  whether  he,  for  example,  is  exalted,  egotistic, 
beyond  criticism,  self-sufficient,  and  believes  that  anything  that  he 
may  decide  to  do  is  justifiable.  It  is  important  to  know  whether 
the  patient  in  his  past  career  has  been  impulsive,  whether  he  has 
shown  tendencies  to  do  unusual,  bizarre,  or  grotesque  things,  or  to 
fly  into  passions,  or  to  be  uncontrollable  from  slight,  inadequate,  or 
unexpected  reasons.  Threats  have  to  be  evaluated  and  an  opinion 
reached  as  to  whether  the  patient  really  means  to  carry  them  out,  or 
whether  they  are  used  as  a  means  of  emotional  catharsis.  The  general 
education,  bringing  up,  and  ideals  of  the  individual  are  important  as 
indicating  what  he  is  liable  to  do.  A  person  who  was  brought  up 
originally  with  a  proper  regard  for  the  proprieties,  who  is  essentially 
a  gentleman  or  a  gentlewoman,  is  by  that  very  token  not  so  apt  to 
commit  some  vulgar,  rowdy,  indecent  act. 

And  finally,  it  may  be  said  that  while  the  general  attitude  toward 
paranoia  has  perhaps  not  materially  changed,  while  there  is  still 
pretty  good  ground  for  believing  that  a  certain  class  to  whom  the  term 
paranoia  is  perhaps  alone  applicable,  are  inaccessible  to  therapeutic 
endeavor  and  are  doomed  to  suffer  from  their  psychosis  throughout 
their  lives,  still  even  if  this  is  so  there  is  no  absolute  way  of  deter- 
mining this  fact  except  by  a  consistent  and  sufficiently  prolonged 
effort  to  modify  the  course  of  the  disease,  and  with  the  several  cases 
already  in  the  literature  which  indicate  that  conditions  that  might 
well  have  been  considered  chronic  and  irrecoverable  if  taken  at  their 
face  value  can  still  be  materially  benefited  and  perhaps  cured,  no  one 
is  in  a  position  to  pass  final  sentence  upon  any  patient  after  an 
examination  or  two,  but  on  the  contrary,  has  the  right  to  feel  that 
there  is  some  hope  for  all  of  them,  and  that  at  least  hope  should  not 
be  abandoned  until  constant  therapeutic  efforts  have  been  applied  for 
a  reasonable  time. 


CHAPTER    XIX. 
EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION. 

Epilepsy,  the  "falling  sickness,"  has  been  known  from  the  earliest 
times,  the  very  word  itself  carrying  in  its  history  (it  is  derived  from  a 
Greek  verb  meaning  "to  seize  upon")  evidences  of  the  animistic 
hypotheses  of  earlier  and  relativel}^  more  primitive  ways  of  thinking. 

The  word  epilepsy  is  used  as  a  symbol  under  which  are  grouped  a 
great  variety  of  conditions  which  in  general  are  characterized  by  sud- 
den and  relatively  transient  attacks  involving  for  the  most  part  dis- 
turbances of  consciousness  ("faints,"  "absences,"  "blanks,"  amnesias) 
and  convulsive  seizures  involving  the  voluntary  and  involuntary 
musculature.  Such  attacks  are  the  outward  manifestations  of  a  wide 
variety  of  conditions  ranging  all  the  way  from  the  so-called  functional 
neuroses  and  psychoneuroses  (hysteria,  compulsion  neurosis) ,  the  more 
frank  psychoses  (dementia  precox),  toxemic  states  (uremia,  alcohol), 
many  organic  brain  diseases  (paresis,  cerebral  syphilis,  abscess,  soften- 
ing and  tumors)  to  the  grosser  defects  of  development  (idiocy). 

The  natural  evolution  of  the  concept  symbolized  by  epilepsy  in  the 
recognition  that  similar  "seizures"  may  result  in  such  a  multiplicity 
of  conditions  has  resulted  in  a  tendency  to  speak  of  "the  epilepsies" 
rather  than  of  "an"  epilepsy  and  makes  it  worth  while  to  consider  the 
attack  as  due  to  a  faulty  distribution  of  energy  which  ma}'  be  brought 
about  in  many  ways  and  through  divers  mechanisms.  The  wide 
variety  of  conditions,  as  a  part  of  whitfh  convulsive  reactions  with 
associated  disturbances  of  consciousness  occur,  cannot  be  too  much 
emphasized.  The  toxic  states  (endogenous  or  exogenous)  are  usually 
transitory  and  depend  upon  the  continuance  of  the  toxemia,  but  in 
defective  parathyroid  functioning  with  disordered  calcium  metabolism 
the  convulsive  phenomena  continue  because  the  underlying  metabolic 
disorder  cannot  be  permanently  relieved.  Marked  organic  changes 
which  are  responsible  for  convulsive  attacks  are  usually  cerebral 
(tumor,  softenings,  hemorrhage,  meningitis),  but  certain  organic 
conditions  resident  elsewhere,  notably  the  cardiopathy  of  Stokes- 
Adams  disease  and  the  condition  resulting  in  animals  from  thymus 
extirpation,  appear  to  be  sufficient  causes.  While  still  more  obscure 
factors  determine  vagal  and  vasovagal,  gastric  and  intestinal  attacks 
which  appear  to  be  dependent  upon  elements  of  constitutional  make  up 
at  the  level  of  the  vegetative  nervous  system.  It  is  worth  while,  there- 
fore, to  attempt  to  get  a  view-point  of  all  these  conditions  dependent 
upon  their  common  element — faulty  energy  distribution. 


668         EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

For  the  purpose  of  comprehending  the  epileptic  phenomena  then, 
the  nervous  system  may  be  viewed  in  a  very  simple  way.  From 
the  stand-point  of  structure  it  may  be  thought  of  as  consisting  of 
receiving  organs,  designed  either  to  come  in  contact  with  the  external 
world  (exteroceptors)  or  with  other  parts  of  the  body  (proprioceptors) 
spoken  of  collectively  as  receptors.  The  combined  material  accumulated 
through  these  receptors  forms  the  basis  upon  which  certain  extensions 
of  the  nervous  system  (effectors)  are  devised,  whereby  the  reactions 
of  the  body  are  conditioned  in  a  way  to  bring  about  that  adaptation 
essential  to  life  or  to  the  maintenance  of  the  social  structure.  In  this 
way  the  nervous  system  is  viewed  as  a  mass  of  interrelated  reflexes 
redistributing  the  energy  received,  for  the  purposes  of  the  organism. 

As  the  incoming  stimuli  are  multitudinous,  so  the  outgoing  activities 
are  correspondingly  diverse,  and  a  healthy  organism  is  able,  by  reason 
of  its  nervous  mechanisms,  to  so  distribute  the  energy  received  as  to 
bring  about  a  series  of  harmoniously  adjusted  activities,  be  they 
physicochemical,  sensorimotor,  or  psychic.  This  view-point,  that 
energy  distribution  takes  place  at  all  of  these  levels,  should  not  be 
lost  sight  of,  as  there  is  a  tendency  to  think  of  the  problem  solely  in 
terms  of  muscular  work.  Atwood's  "man  in  the  box"  broke  up  more 
nitrogen  compounds  during  mental  than  during  mechanical  work. 
This  breaking  up  of  nitrogen  compounds  is,  however,  only  one  form 
of  registering  the  energy  output. 

That  which  characterizes  a  well-adjusted  nervous  mechanism  is  its 
ability  to  properly  and  in  an  orderly  manner  distribute  its  energy, 
but  that  which  characterizes  the  pictures  of  the  disorders  included 
in  this  chapter  is  inefficiency  in  this  regard  which  may  be  more  espe- 
cially emphasized  at  the  physicochemical,  the  vital,  or  the  psychic 
levels. 

An  explanation  for  epileptic  attacks  which  finds  its  ultimate  expres- 
sion under  such  symbols  as  eye-strain,  floating  kidney,  gliosis,  or  like 
specific  indictments  fails  to  realize  that  the  nervous  system  contains 
representations  of  all  of  the  organs  and  that  the  final  activity  of  the 
human  body  is  the  result  of  the  balance  which  has  been  struck  among 
innumerable  tendencies.  The  part  that  any  particular  organ  plays 
can  only  be  understood  when  taken  in  consideration  with  the  organism 
in  its  totality  and  realizing  the  specific  part  that  the  organ  in  question 
plays  in  the  whole  problem. 

Bearing  in  mind  this  view  of  the  nervous  system,  as  a  great  mass  of 
complexly  interrelated  reflexes,  and  further,  the  law  of  avalanche 
(Cajal)  which  insures  the  continuous  breaking  up  of  the  original 
sensory  stimulus  into  an  ever-increasing  number  of  avenues  of  dis- 
charge, it  will  be  seen  how  many  ways  are  open  to  interfere  with  the 
orderly  procession  of  energy  through  this  complicated  series  of  reflex 
arcs.  The  nature  of  the  epileptic  discharge,  essentially  a  manifesta- 
tion of  energy  at  greatly  reduced  adaptive  efficiency,  and  the  destruc- 
tive character  of  certain  pathological  lesions  which  initiate  it  (impaired 


EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION     669 


metabolism,  gross  destructions,  psychic  imbalances)  indicates  that  the 
essential  defect  is  not  irritant  but  destructive,  the  blocking  or  closing 
of  man}^  paths  of  outlet  structur- 
ally or  by  inhibition,  and  so  accu- 
mulating the  discharge  within 
relatively  narrow  confines.  Such 
a  conception  would  apply  equally 
well  to  the  "idiopathic"  or 
"genuine  epilepsy"  with  Am- 
mon's  horn  ghosis  and  to  the  epi- 
lepsy associated  with  marked 
developmental  defects  (idiocy) 
in  which  it  may  be  conceived 
that  the  wider  paths  for  avalanche 
discharge  have  not  been  laid 
down.  This  view-point  is  also 
consistent  with  the  different 
levels  at  which  the  discharge 
may  take  place — psychic,  phy- 
siological, and  physicochemioal 
— the  character  of  the  attack  as 
limited  to  certain  levels,  the  psy- 
chic (hysteria,  dementia  precox), 
the  physiological  (Jacksonian 
types),  the  physicochemioal  (tetany);  the  distribution  of  the  dis- 
charge, general  attacks  ("genuine  epilepsy"),  localized  attacks  (Jack- 


FiG.  305. — Epileptic,  showing   scars   over 
eyebrows  from  falls. 


Fig.  306. — Epileptic,  showing  scar  from  burn  due  to  injury  during  convulsion. 


670         EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

sonian  types),  and  as  being  initiated  by  sensory,  motor,  or  psychic 
prodromes. 

Symptom  Groups. — Convulsions  may  occur,  as  already  indicated,  in 
a  great  variety  of  conditions  while  distinctly  explosive  attacks,  not 
convulsive  in  the  sense  of  muscular  spasms,  occur  under  still  wider 
conditions  in  states  not  definitely  epileptic  but,  with  reference  to  the 
more  essential  epilepsies,  in  what  may  be  referred  to  as  borderland 
conditions. 

The  essential  epilepsies  have  been  divided  into  the  late  epilepsies 
occurring  relatively  late  in  life  and  dependent  upon  toxemias  and 
gross  organic  changes  and  the  early  epilepsies  which  occur  relatively 
early  in  life,  generally  before  or  during  adolescence. 

Epilepsies  of  Gross  Brain  Disease. — These  occur  in  paresis,  cerebral 
syphilis,  brain  cysts  (echinococcus,  etc.),  hydrocephalus,  the  cerebral 
meningitides  (syphilitic,  tuberculous,  serous,  and  pachymeningitis), 
bony  tumor  of  skull,  traumatisms  (fractures,  insolation,  concussions, 
hemorrhages),  multiple  sclerosis,  cerebral  sclerosis  and  gliosis,  cerebral 
arteriosclerosis,  the  encephalitides  and  myoclonias. 

The  Epilepsies  of  Toxic  and  Infectious  Origin. — ^The  toxemias  include 
those  of  endogenous  origin  (uremia,  diabetes),  of  exogenous  origin 
(metallic — such  as  lead  and  arsenic,  and  strychnin,  alcohol,  and  carbon 
monoxide) . 

The  infections  are  more  especially  the  exanthemata,  influenza, 
rabies,  malaria,  rheumatism,  syphilis,  malaria,  etc.,  operating  either 
through  the  mechanism  of  an  overwhelming  toxemia  or  by  meningi- 
tides or  encephalitides. 

Anomalous  and  Borderland  Conditions. — Here  are  included  certain 
internal  secretory  imbalances,  particularly  diseases  of  the  thymus, 
thyroid,  and  parathyroids  and  degeneratio-adiposo-genitalis. 

Certain  high  level  attacks  occur  in  hysteria,  compulsion  and  anxiety 
neuroses,  and  in  dementia  precox,  especially  the  catatonic  form. 

Here  also  should  be  included  the  vagal^  and  vasovagal  attacks,  slight 
passing  disturbances  of  consciousness  associated  with  vertigo  and 
sometimes  sensory  disorders  as  loss  of  sight,  possibly  certain  sensory 
disorders  of  fulminating  character  such  as  migraine,  and  some  disturb- 
ances of  sleep  (narcolepsy)  disturbances  of  consciousness  of  syncopal 
nature,  and  the  affect  epilepsies  of  the  Bratz  type. 

Pathological  Groups. — ^The  preceding  clinical  grouping  gives  a  fair 
idea  of  the  pathological  conditions  which  may  be  found.  The  follow- 
ing grouping  is  given  by  Alzheimer^  as  a  result  of  the  histological 
examinations  of  63  cases.  These  simply  indicate  the  reasons  why 
and  how  the  structure  of  the  brain  is  modified  thus  changing  its 
functional  capacity  as  an  energy  distributor. 


1  Wm.  R.  Gowers,  The  Borderland  of  Epilepsy,  Philadelphia,  1907. 

2  Alzheimer    and    Vogt,    Die    Gruppierung    der     Epilepsie,    Jahresversammlung    des 
deutschen  Vereins  fiir  Psychiatric,  1907;  Ref.  AUg.  Zeitschr.  f.  Psych.,  Bd.  Ixiv,  1907. 


CLASSICAL  EPILEPSY  671 

A.  Cases  with  very  obscure  etiology  (genuine  epilepsy) : 

1.  This  group  comprises  60  per  cent,  of  the  cases: 

(a)  With  sclerotic  changes  in  Amnion's  horn. 

(b)  With  superficial  gliosis  of  the  hemispheres. 

(c)  With  signs  of  an  acute  process  (status)  besides  a  and  b. 

B.  Cases  due  to  external  poisons: 

1.  Alcohol:    Different  anatomical  changes,  as  in  chronic  alco- 

holism.    Besides  these  sometimes  acute  changes,  as  in 
delirium. 

2.  Lead:    Different  changes.     Experimentally  lead  produces 

a  genuine  encephalitis. 

C.  General  diseases : 

1.  Syphilis:    Different  forms  of  brain  syphilis,  especially  the 

endarteritis  of  the  finer  vessels  (Nissl,  Alzheimer). 

2.  Arteriosclerosis. 

D.  Focal  diseases:     Most  of  the  cases  in  this  group  are  cases  of 

epilepsy  with  idiocy  after  encephalitis. 

E.  Arrests  of  development: 

1.  Stadium  verrucosum  (Rancke). 

2.  Sclerosis  tuberosa. 

This  survey  will  suffice  to  show  what  a  wide  variety  of  conditions 
have  been  included  under  the  term  epilepsy  and  also  to  point  out  the 
various  groups  that  are  being  at  present  split  off  and  separately  iden- 
tified. What  has  been  said  about  the  distribution  of  energy  applies 
to  the  broad  group  of  convulsive  reactions.  The  disease  "genuine  epi- 
lepsy" will  now  be  briefly  described,  although  it  is  extremely  difficult 
to  do  this  at  all  accurately  as  it  must  be  borne  in  mind  that  hereto- 
fore all  matters  of  description  and  questions,  such  as  those  of  heredity, 
are  with  practical  uniformity  considered  with  reference  to  "epilepsy," 
without  eftort  at  discrimination,  much  as  is  the  case  with  the  corre- 
sponding conglomerate  "insanity." 

CLASSICAL   EPILEPSY 

This  roughly  corresponds  to  the  group  "genuine  epilepsy"  of 
Alzheimer. 

Heredity. — The  genuine  epileptic  usually  comes  from  a  badly 
tainted  stock.  Epilepsy  may  not  appear  in  the  ancestors  but  they 
and  often  the  collaterals  show  evidences  of  ill-defined  nervous  disorders 
(according  to  Davenport  and  Weeks,^  migraine,  chorea,  paralysis,  and 
extreme  nervousness).  Epilepsy  and  feeble-mi ndedness  show  a  great 
similarity  in  their  hereditary  reactions  and  both  appear  to  be  due  to 
a  defect  of  the  germ  plasm,  that  is,  they  are  both  recessives.  As 
should  be  expected,  therefore,  the  two  conditions  are  frequently 
found  associated.     This  is  also  of  significance  in  relation  to  what 

1  A  First  Study  of  Inheritance  in  Epilepsy,  Journal  of  Nervous  and  Mental  Disease, 
1911,  vol.  xxxviii,  No.  11. 


672         EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

has  already  been  said  about  the  developmental  failure  to  lay  down  the 
paths  for  the  higher  avenues  of  sensory  avalanche. 

The  Epileptic  Constitution. — -This  type  of  epileptic  is  apt  to  be 
morose,  irritable,  suspicious,  and  hypochondriacal.  He  is  quite  char- 
acteristically unreliable  and  with  it  all  frequently  presents  a  very 
aggressive  form  of  sentimental,  shallow  religiosity.  This  type  of  epi- 
leptic, in  general  then,  is  very  sensitive,  irritable  and  insincere.  He 
is  egocentric  to  a  very  considerable  degree,  paying  great  attention  to 
himself,  his  own  feelings,  his  state  of  health,  his  physical  comforts, 
and  his  immediate  surroundings.  Jlis  interests  are  variable  and  he 
presents  light  variations  of  mood  with  perhaps  headache  and  a  ten- 
dency generally  to  hypochondriacal  fixations.  His  interests  all  tend 
to  be  concentrated  in  this  egocentric  constellation.  His  reactions 
of  irritability  and  unreasonableness  present  infantile  characteristics. 
Many  epileptics  are  feeble-minded  or  more  profoundly  defective,  and 
rather  in  conformity  with  this  frequent  finding  the  word  associations 
from  epileptics  have  close  analogies  to  the  word  associations  of  the 
imbecile.  In  addition  to  these  traits  of  character  these  epileptics  are 
usually  lazy,  frequently  they  lie  openly,  present  an  attitude  based  on 
high  moral  standards  of  great  respect  and  consideration  to  one's  face 
and  quite  the  opposite  when  one's  back  is  turned.  Their  general 
health  is  apt  to  be  good  and  they  often  have  enormous  appetites,  and 
are  especially  fond  of  proteids.  While  good-natured,  even-tempered, 
well-disposed  epileptics  exist  they  are  more  apt  to  be  most  difficult 
problems  to  get  along  with,  and  as  a  'class  in  the  hospital  they  are 
extremely  difficult  to  care  for.  Passing  attacks  of  mental  disturbance 
occur  in  the  interparoxysmal  period  without  apparent  relation  to 
seizures.  Attacks  of  transitory  ill-humor,  according  to  Aschaffenberg, 
occur  in  78  per  cent,  of  cases.  This  is  a  condition  of  irritability,  un- 
reasonableness, sometimes  associated  with  delusions  and  hallucinations. 
The  patient  is  in  a  "touch-me-not"  state  and  very  apt  to  get  into 
quarrels  or  make  attacks.  Rarely  the  disturbance  is  expansive  in  type 
and  in  these  c^ses  may  be  associated  with  religious  fervor. 

A  study  of  the  sexual  characteristics  of  epileptics  has  recently  been 
made  by  Maeder.^  As  a  result,  he  finds  the  sexuality  of  the  epileptic 
still  largely  undeveloped,  still  only  little  removed  from  the  infantile 
stage.  The  sexual  feelings  are  very  prominent  and  are  aroused  in 
many  ways:  autogenically,  constituting  auto-erotism,  and  giving  rise 
to  such  phenomena  as  masturbation,  and  by  stimuli  from  without, 
constituting  allo-erotism,  which  gives  rise  to  a  normal  libido  (hetero- 
sexuality),  homosexuality,  exhibitionism,  etc.  Maeder  uses  the  term 
polyvalent  to  describe  this  characteristic  of  the  epileptic  sexuality  which 
permits  it  to  be  aroused  by  many  kinds  of  excitants. 

The  epileptic  state  leads  in  a  certain  proportion  of  cases,  if  it  has 
begun  in  early  life,  to  conditions  of  feeble-mindedness,  imbecility,  and 

1  Sexualitat  und  Epilepsie,  Jahrb.  f.  Psychoanalytische  u.  Psychopathologische  Fors- 
chungen,  1909. 


< 


CLASSICAL  EPILEPSY  673 

idiocy,  or,  depending  upon  the  same  causes,  is  associated  with  these 
conditions.  Epilepsy  tends,  in  many  cases,  to  produce  a  general  mental 
deterioration  {epileptic  dementia)  which  may  become  very  profound. 

A  recent  study  of  the  personality  of  epileptics  by  Clark^  has  led  him 
to  the  conclusion  that  this  general  type  of  character  as  described  above 
is  a  result  of  the  disease  and  not  a  precedent  condition. 

Scripture  and  Clark^  have  described  the  epileptic  voice  sign  and  found 
it  in  75  per  cent,  of  cases.  The  voice  has  been  studied  by  the  "air 
puff"  method  of  recording  on  the  kymograph.  A  measure  of  the  wave 
gives  the  rates  of  vibration.  A  line  connecting  the  tops  of  the  ordinates 
produces  the  "melody  plot."  Normally  each  vowel  has  a  rising  and 
falling  melody.  In  epilepsy  the  vowels  run  along  on  an  even  tone — 
"plateau  speech."    This  is  very  characteristic  and  easily  recognized. 

Muskens,  in  his  study  of  the  muscular  phenomena,  has  found 
fatigability  and  weakness  of  single  muscles  or  muscle  groups,  startings, 
shocks  and  cramp-like  contractions  especially  just  before  or  after  going 
to  sleep.  Gastro-intestinal  and  vasomotor  disturbances  have  also  been 
noted. 

The  Seizure. — ^The  classical  major  epileptic  attack  (grand  mal)  is 
sudden  in  onset,  often  preceded  by  a  warning — aura.  The  patient 
falls  and  the  attack  immediately  develops  into  a  tonic  spasm  with 
unconsciousness.  The  tonicity  is  replaced  in  a  few  moments  by  clonic 
spasms  which  gradually  subside.  There  is  then  often  a  short  period 
of  automatic  activity  followed  by  a  gradual  return  to  full  conscious- 
ness, or  the  patient  sinks  at  once  into  a  deep  sleep  from  which  he 
awakes  complaining  of  lameness  and  weakness  in  the  muscles  that 
were  convulsed,  and  perhaps  headache. 

The  attack  has  been  described  by  authors  in  great  detail,  but  is 
really  different  in  practically  each  case,  although  the  type  tends  to 
remain  the  same  in  each  patient. 

The  aura  may  be  sensory,  motor,  or  psychic.  The  sensory  warnings 
may  occur  in  any  of  the  sensory  fields:  the  visual  (flashes  of  light, 
hallucinations),  the  olfactory  (odors,  usually  bad — uncinate  fits),  the 
auditory  (simple  sounds  or  hallucinatory  voices),  etc.  The  epigastric 
aura  is  most  common  and  consists  of  a  wide  variety  of  disagreeable 
sensations  in  the  epigastrium. 

The  muscles  first  involved  vary  greatly,  as  does  also  the  order  in 
which  they  are  involved — march  of  the  convulsion.  The  patient  falls 
at  the  beginning  of  the  spasm,  the  direction  of  the  fall  being  dependent 
upon  the  muscles  first  affected.  In  a  few  moments  all  of  the  voluntary 
muscles  are  convulsed,  including  the  muscles  of  respiration,  producing 
cyanosis,  and  the  jaw  muscles,  resulting  in  biting  the  tongue.  With 
the  beginning  of  the  clonic  stage  frothy,  bloody  saliva  issues  from  the 
mouth  and  the  cyanosis  gradually  disappears  with  the  resumption  of 

•  A  Personality  Study. of  the  Epileptic  Constitution,  Amer.  Jour.  Med.  Sci.,  November, 
1914. 
^  Researches  on  the  Epileptic  Voice,  Proc.  New  York  Neurol.  Soc,  November  12,  1907. 
43 


674         EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

respiration.  Urine  and  feces  may  be  passed  during  the  attack — usually 
only  the  former.  In  the  partial  or  incomplete  seizures — petit  mal — the 
convulsive  phenomena  are  much  milder  and  may  even  escape  observa- 
tion altogether,  while  the  disturbance  of  consciousness  is  shorter  in 
duration  and  less  profound.  The  patient  may  blanch,  become  confused 
for  a  few  moments,  perhaps  falter  in  what  he  is  doing,  or  fumble  for  a 
few  moments  about  his  clothing  in  a  dazed  fashion  and  then  go  on 
about  his  affairs  as  if  nothing  had  happened.  These  attacks  are  also 
often  preceded  by  an  aura. 

The  psychic  disturbances  associated  with  the  attack,  before  and 
after,  or  replacing  it  are  many  and  varied. 

In  a  great  many  epileptics  there  is  a  marked  disturbance  preceding 
the  convulsion,  sometimes  of  several  days'  duration,  and  those  who  are 
accustomed  to  the  patient  can  tell  that  a  fit  is  impending.  This  change 
manifests  itself  in  increased  irritability,  complaining,  sometimes  by 
depression  or  dulness,  and  there  may  be  associated  disturbances  of  the 
sensorium,  hypochondriacal  complaints  and  hallucinations.  All  these 
conditions  are  commonly  promptly  relieved  by  the  fit. 

Immediately  after  the  convulsion  there  is  often  a  temporary  condi- 
tion of  confusion.  The  patient  rises  clumsily,  looks  about  him  in  a 
bewildered  manner  and  often  does  some  semiautomatic  acts,  such 
as  taking  off  his  clothes.  Also  following  the  attack  a  transitory 
exhaustion  paralysis  in  the  overacting  muscles  makes  itself  apparent. 

Just  before,  or  more  commonly  after,  the  convulsion  a  condition  of 
active  excitement  may  occur  which  may  reach  the  stage  of  frenzy. 
In  this  state  the  patient  is  a  veritable  wild  man — epileptic  furor.  He 
is  liable  to  kill  anyone  who  approaches  or  even  himself.  Fortunately 
his  efforts  are  diffuse  and  not  coherently  directed.  During  this  attack, 
which  is  usually  brief,  he  has  to  be  restrained  and  at  the  end  is  quite 
completely  exhausted. 

An  attack  of  mental  disturbance  may  take  the  place  of  the  convulsion 
and  thus  become  an  epileptic  equivalent.  These  attacks  of  psychic 
epilepsy  frequently  take  the  form  of  so-called  epileptic  automatism  or 
epileptic  dream  states.  In  these  conditions  the  patient  may  do  almost 
anything  and  when  he  comes  to  himself  he  has  absolutely  no  recollec- 
tion of  what  has  happened.  Usually  the  attacks  are  of  short  duration 
and  the  acts  rather  simple — more  simple  than  in  the  dream  states  of 
alcohol  or  hysteria.  However,  they  may  last  for  days,  all  sorts  of 
things  may  be  done,  crimes  may  even  be  committed,  so  that  the  con- 
dition often  becomes  of  great  medicolegal  importance.  The  crimes  of 
violence  are  often  noted  for  their  ferocity  and  brutality. 

It  must  not  be  forgotten  that  these  states  may  be  associated  with  a 
seizure  that  was  so  slight  as  not  to  have  been  noticed.  Evidences  of 
such  a  seizure,  especially  in  medicolegal  cases,  should  always  be 
looked  for. 

Transitory  conditions  of  depression,  excitement,  confusion,  delirium, 
and  stupor  may  develop  and  quite  characteristically  a  condition  of 


CLASSICAL  EPILEPSY  675 

ecstasy  with  hallucinations.  The  patient  sees  the  gates  of  Heaven  open 
and  as  the  heavenly  hosts  appear  he  hears  himself  addressed  by  the 
voice  of  God. 

The  transitory  states  of  ill-humor,  as  described  by  Aschaffenberg 
in  the  interparoxysmal  state,  might  also  be  considered  as  psychic  equiv- 
alents; these  are  frequently  associated  with  drinking. 

Besides  these  conditions,  'paranoid  psychic  states  are  quite  common, 
while  of  the  more  transitory  psychic  manifestations /w^we-s  are  frequent 
and  certain  types  of  dipsomania  appear. 

In  addition  to  the  symptoms  thus  far  indicated  various  observers 
have  found  e^ddences  of  an  altered  blood  picture  such  as  leukocytosis 
and  hypo-eosinophilia,  while  disorders  of  metabolism  with  hyper- 
toxicity  of  the  secretions,  has  long  been  adduced  as  proof  that  the 
manifestations  were  dependent  upon  chemical  poisons  due  to  faulty 
metabolism. 

Meaning  of  the  Attack. — ^Bearing  in  mind  what  has  already  been  said 
about  the  distribution  of  energy  it  will  be  of  advantage  to  pursue  this 
line  of  thought  somewhat  further. 

Energy  flow  may  be  blocked,  dammed  up,  and  break  through  in 
diffuse  discharge  at  any  level — ^psychic,  sensorimotor,  or  physico- 
chemical.  This  being  so  it  would  be  expected,  as  is  the  case,  as  already 
indicated,  to  find  disturbances  at  each  of  these  levels.  In  the  classical 
epileptic  attack  all  these  levels  are  involved,  but  what  is  of  equal  or 
greater  significance  to  the  general  hypothesis  is  that  there  are  attacks 
practically  limited  to  one  level,  and  a  study  of  the  several  types  of 
convulsive  reaction  will  show  a  series  of  cases  reaching  through  all 
of  the  intermediate  stages  from  the  highest  to  the  lowest  instinctive 
levels. 

The  hysterical  convulsion  offers  an  example  of  a  high  level  convul- 
sive type  of  reaction.  This  seizure  is  admittedly  psychogenic  in  origin 
and  presents  the  picture  of  a  conversion  of  psychological  into  physio- 
logical symbols.  In  other  words,  the  patient  escapes  from  his  painful 
ideas  by  converting  them  into  physical  symptoms.     (See  Hysteria.) 

The  disturbance  of  consciousness  in  these  hysterical  attacks  is 
relatively  slight,  much  less  than  it  outwardly  appears  to  be,  while  the 
whole  situation  is  quite  near  the  surface  and  with  very  little  effort 
can  be  brought  to  conscious  control. 

Next  lower  in  the  scale  of  levels  are  the  psychasthenic  convulsions 
of  Oppenheim  (compulsion  neurosis  type)  which  are  expressions  of  a 
more  severe  grade  of  neurosis  but  still  within  strictly  psychological 
levels. 

Then  come  the  very  interesting  affect  epilepsies  of  Bratz  and  Leu- 
buscher.  These  are  distinctly  epileptoid  types  of  reaction  conditioned 
by  purely  psychological  situations.  Here  the  outward  semblance  to 
a  deeper  level  epilepsy  is  much  greater  but  the  situation  is  still  a 
psychological  one.  The  reaction  of  the  patient  here  is  to  conditions 
that  are  absolutely  intolerable  and  to  which  no  adjustment  is  possible, 


676         EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

such  a  situation,  for  example,  as  a  young  man  has  to  confront  when  the 
key  is  turned  upon  him  and  he  is  called  upon  to  realize  that  he  is  in 
prison  with  a  life  sentence  to  face.  Under  these  circumstances  the 
patient  may  become  a  veritable  "wild  man,"  beat  his  clenched  fists 
against  the  bars,  rush  aimlessly  about  destroying  clothes  and  bedding, 
and  beat  his  head  against  the  walls  in  ineffectual  attempts  at  self- 
destruction.  Hallucinatory  disturbances  may  accompany  these  attacks 
and  amnesia  follow  them,  though  consciousness  during  the  attack  is 
not  entirely  lost.  That  these  patients  are  much  more  seriously  bur- 
dened constitutionally  and  more  nearly  allied  to  "genuine  epilepsy" 
than  the  psychasthenic  types  of  Oppenheim  is  indicated  by  the  fact 
that  they  give  a  history  of  "fits"  in  childhood  while  the  psychasthenic 
types  show  tics,  phobias,  and  compulsions. 

In  the  classical  epileptic  seizure  the  greater  severity  and  seriousness 
of  the  attack  is  indicated  by  the  complete  loss  of  consciousness  and  the 
still  further  reduction  in  the  purposeful  and  coordinated  adjustment 
of  the  muscular  reactions.  These  have  now  become  utterly  disorgan- 
ized. The  attack  has  involved  far  more  than  the  psychological  levels 
and  included  the  sensorimotor  and,  as  indicated  by  the  toxicity  of 
the  excretions,  the  biochemical. 

The  low  instinctive  level  to  which  the  epileptic  is  reduced  by  his 
seizure  can  be  appreciated  by  observing  his  activities  as  he  is  "coming 
out"  of  the  attack.  His  respiration  is  at  first  distinctly  abdominal 
(infantile  type),  he  makes  characteristic  sucking  movements  with  his 
lips,  and  his  movements,  from  the  complete  disorganization  into  which 
they  have  been  thrown,  assume  at  first  an  aimless  fumbling  with  his 
clothes,  a  tentative  feeling  about  as  he  instinctively  tries  to  readjust 
himself  to  reality,  to  "find  himself"  again.  In  this  tentative  "feeling 
about"  he  repeats  in  a  few  minutes  the  process  of  relating  himself  to 
reality  which  is  a  normal  period  of  development  in  the  child. 

Ferenczi^  has  endeavored  to  classify  the  neuroses  with  reference  to 
the  stage  of  development  they  represent  and  in  accordance  with  this 
scheme  suggests  that  epilepsy  belongs  to  the  period  of  wish-fulfilment 
by  means  of  incoordinate  movements.  It  is  known  how  some  children 
when  thwarted  will  cry  out,  thrash  about  and  sometimes  straighten 
out  rigidly,  "  lose  their  breath"  and  become  blue.  Attacks  of  "  temper" 
the  mother  calls  them.  Later  on  the  child  will  kick  the  chairs  and  tear 
up  its  books  under  similar  circumstances  while  regression  to  approxi- 
mately the  same  level  is  shown  when  an  adult  stamps  his  foot,  clenches 
his  fists,  grinds  his  teeth,  and  otherwise  shows  reactions  of  anger  which 
are  quite  ineffectual  to  efi^ect  any  change  whatever  in  conditions.  The 
meaning  of  it  all  is  an  absolute  inability  to  accept  or  to  adjust  and  an 
equally  determined  attitude  that  it  is  not  so  because  it  just  cannot  be 
so.  An  effort  to  force  circumstances  to  be  different  by  a  supreme  effort 
of  thinking  them  different  which  when  it  fails  results  in  a  flight  from 

1  Entwicklungsstufen  des  Wirklichkeitssinnes,  Internationale  Zeitschr.  f.  Aerztliche 
Psychoanalyse,  1913,  vol.  i. 


CLASSICAL  EPILEPSY  677 

the  whole  thing  into  the  rigidity  and  unconsciousness  of  the  epileptic 
seizure. 

This  way  of  viewing  the  epileptic  attack  is  warranted  because  it 
follows  along  a  path  that  proceeds  from  the  known  to  the  unknown. 
The  mechanism  at  the  higher  psychological  levels  can  be  worked  out 
and  although  those  at  lower  levels  cannot  it  would  appear  that  the  two 
extremes  are  connected  by  a  regular  series  of  intermediate  stages  as 
represented  in  types  of  cases.  It  can  only  be  assumed  that  the  disorder 
in  "genuine  epilepsy,"  has  to  do  with  adjustments  at  deep  instinc- 
tive levels,  adjustments  that  are  profoundly  biological  in  character, 
and  that  the  conflict  may  almost  threaten  life  itself  in  order  to  under- 
stand the  severity  and  seriousness  of  the  attack  as  a  reaction  to  failure. 
A  study  of  the  aura  as  a  clue  to  the  point  at  which  blocking  of  energy 
commences,  and  a  detailed  analysis  of  all  the  elements  of  the  attack 
plus  an  analysis  of  the  make-up  of  the  individual  in  the  interparoxysmal 
period  would  seem  to  offer  the  mode  of  approach  to  a  further  under- 
standing of  the  mechanisms  in  individual  cases.  The  depth  to  which 
such  an  analysis  would  have  to  go  and  the  severity  of  the  constitu- 
tional burden  in  the  classical  types  of  the  disease  is  indicated,  for 
example,  by  the  prevalence  of  the  epigastric  aura  the  characteristics 
of  which  indicate  the  possibility,  at  least,  that  the  disorder  reached 
as  deep  as  the  vegetative  nervous  system  level. 

It  can  be  seen  from  this  discussion  why  the  epileptic,  burdened  by 
deeply  instinctive  defects  of  biological  adjustment,  should  dement. 
It  is  also  consistent  with  this  view  that,  in  general,  this  should  not  be 
true  of  symptomatic  epilepsy.  A  localized  lesion  of  the  cortex,  for 
example,  may  produce  convulsive  reactions  in  well-defined  groups  of 
muscles  only  without  loss  of  consciousness  (Jacksonian  type).  Here 
there  is  no  defect  of  biological  adjustment  involving  the  individual  as 
such.  A  group  of  muscles  only  has  been  cut  off  from  effective  associa- 
tional  relationship  with  higher  levels  and  so  becomes  reduced  in  its 
possibilities  of  reaction  to  relatively  incoordinate,  automatic  and 
purposeless  types.  A  portion  only  of  the  machinery  has  been  damaged, 
the  individual  remains  otherwise  intact.  The  disorder  is  confined  to 
the  sensorimotor  level. ^ 

From  this  point  of  view  it  seems  that  the  toxicity  of  the  blood  and 
urine  is  only  an  outward  evidence  of  the  depth  of  the  disorder  rather 
than  an  indication  of  its  cause.  It  is  true  that  certain  toxic  substances 
do  produce  convulsions,  but  they  do  it  by  damaging  the  machinery 
like  the  cortical  lesions  just  referred  to  and  the  seizures  cease  with 
the  removal  of  the  poison.  This  is  seen  in  severe  forms  of  infection, 
febrile  states  in  children,  alcohol,  uremia,  etc. 

It  is  interesting  and  significant  to  note,  in  this  connection,  that  the 
form  of  dementia  precox  in  which  convulsive  seizures  are  most  prone 

'  See  Clark,  L.  P.,  Nature  and  Pathogenesis  of  Epilepsy,  N.  Y.  Med.  Jour.,  February, 
1915  et  seq,  for  a  complete  statement  of  this  viewpoint  with  numerous  clinical  proofs 
of  its  value. 


678         EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

to  occur  is  the  catatonic.  In  the  other  forms  types  of  compromise 
formation  take  place  while  the  catatonic  endeavors  to  cut  out,  to 
enucleate,  so  to  speak,  a  certain  portion  of  his  psyche,  a  condition  much 
more  favorable  to  blocking. 

Varieties  of  Convulsive  Attack.— The  Jacksonian  type  has  already 
been  referred  to  as  has  also  the  distinction  between  the  grand  and 
petit  mal  attacks  and  various  equivalents  (larvated  or  masked  epilepsy). 

In  addition  to  isolated  seizures,  there  are  serial  attacks  in  which 
several  seizures  follow  each  other  at  relatively  short  intervals  and 
finally  status  epilepticus  in  which  large  numbers  of  attacks  accumulate, 
following  each  other  at  short  intervals  until  unconsciousness  becomes 
continuous,  the  attacks  then  merging  into  one  another — overlapping. 
The  temperature  rises  in  this  condition,  life  is  threatened  and  indeed  it 
is  the  typical  mode  of  death  of  the  epileptic  and  his  ever-present  danger. 
Serial  attacks  are  usually  grand  mal,  but  may  be  petit  mal,  while 
status,  even  if  it  begins  as  petit  mal,  soon  takes  on  the  seriousness  of 
the  major  variety.  Psychic  seizures  may  also  be  serial  and  status 
attacks  are  possible  without  or  with  very  minor  convulsive  manifesta- 
tions. Status  may,  and  frequently  does,  develop  in  tjie  symptomatic 
epilepsies  due  to  gross  lesions  of  the  brain  as  well  as  forming  a  frequent 
termination  of  the  partial  or  incomplete  seizures  of  the  Jacksonian  type. 

Myoclonus  epilepsy  appears  to  be  an  association  of  myoclonia  and 
epilepsy.    It  would  seem  that  the  myoclonic  shocks,  however,  gradually 
eventuated  by  a  process  of  summation  into  an  epileptic  seizure.    Some 
epileptics  have  myoclonic  shocks  between  their  attacks  which  appear 
to  be  forerunners  of  the  seizure  rather  than  true  forms  of  myoclonia. 

Continuous  epilepsy. — Similar  to  the  myoclonic  varieties  are  the 
polyclonia  epileptoides  continua  of  Choroschko  and  the  epilepsia 
corticalis  continua  of  Koshewnikow.  This  is  a  condition  of  continuous 
myocloniform  shocks  in  single  muscle  groups,  usually  unilateral  and 
without  loss  of  consciousness. 

Diagnosis. — From  what  has  been  said  it  can  be  seen  that  epilepsy 
is  not  an  entity,  that  the  term  includes  a  great  multitude  of  widely 
different  conditions  and  that  the  problem  of  diagnosis  is  therefore 
the  problem  of  differentiating  the  particular  one  of  these  several 
possibilities  in  an  individual  case.  Diagnosis,  however,  does  not  mean 
giving  a  name  to  a  thing,  but  understanding  it.  Every  individual 
epileptic  should  be  accepted  as  an  individual  problem  and  although  it 
may  often  be  quite  impossible  to  fathom  the  mechanisms  involved,  it 
is  only  by  such  a  method  of  approach  that  anything  worth  while  can 
be  hoped  for  when  the  question  of  treatment  comes  to  be  considered. 

The  possibility  of  exclusively  nocturnal  Sittack.s— nocturnal  epilepsy 
— should  be  borne  in  mind.  It  is  suspicious  if  the  patient  awakes 
tired  and  lame,  as  if  his  muscles  had  been  beaten,  particularly  if  he  shows 
conjunctival  ecchymoses,  a  wounded  tongue,  and  flecks  of  blood  on  the 
pillow.  A  localized  muscular  weakness  that  passes  off  promptly  would 
add  certainty  to  the  diagnosis. 


CLASSICAL  EPILEPSY  670 

Treatment. — The  only  eflScient  prophylaxis  is  not  to  transmit  the 
defective  germ  plasm.  It  would  seem  that  in  the  purely  symptomatic 
epilepsies,  such  as  those  due  to  cortical  traumatism,  that  the  germ  plasm 
might  escape  indictment,  but  this  does  not  necessarily  follow.  A 
certain  proportion  of  these  cases  will  be  found  to  have  had  convulsions 
in  infancy  so  that  they  might  have  been  considered  as  potential  epi- 
leptics predisposed  to  react  by  convulsion-producing  mechanisms. 
This  possibility  is  emphasized  by  the  frequency  with  which  the  symp- 
tomatic epilepsies  develop  status  attacks. 

Treatment  of  the  Attack. — Once  the  attack  has  started  it  is  essential 
to  so  care  for  the  patient  during  his  period  of  helplessness  that  he  may 
not  be  injured  in  any  way.  If  he  has  fallen  in  a  safe  place  he  may  be 
allowed  to  remain  there,  perhaps  only  removing  him  from  proximity  to 
furniture  or  the  wall  against  which  his  limbs  might  be  injured  as  they 
are  in  the  throes  of  the  convulsive  seizure.  The  clothing  should  be 
loosened  about  the  neck  to  permit  free  breathing,  and  if  possible  a 
towel  end  rolled  up  and  pressed  between  the  teeth  to  prevent  injury 
to  the  tongue.  As  a  rule,  he  should  be  permitted  to  remain  on  his  back 
or  side,  according  to  the  position  the  contracted  muscles  force  upon 
him.  A  wound  received  in  falling  may  need  care  and  a  broken  limb 
needs  protection  from  the  severity  of  the  convulsive  contractions  to 
prevent  additional  injury  by  the  broken  ends.  Epileptics,  in  general, 
should  not  be  permitted  to  sleep  unobserved  or  alone  for  fear  they  may 
roll  over  and  smother  during  an  attack.  If  vomiting  occurs  the  patient 
should  be  rolled  on  his  side  and  care  should  be  exercised  to  prevent 
aspiration  of  the  vomitus.  In  the  automatic  period  following,  watchful 
care  is  needed,  but  direct  efforts  at  control  should  be  avoided  if  pos- 
sible as  they  are  not  understood  and  may  only  excite  antagonism. 
The  patient  should  not  be  permitted  to  get  up  until  it  is  seen  that  no 
bones  are  broken.  In  this  automatic  state  an  attempt  to  walk  on  a 
broken  leg  might  easily  compound  the  fracture  for  consciousness  is 
so  reduced  that  pain  would  not  be  felt  or  reacted  to. 

Treatment  of  Status. — Serial  attacks  indicate  possible  danger  of 
status  and  sedatives  should  be  used  to  control  their  frequency,  while 
sudden  withdrawal  of  bromides  should  be  avoided  as  tending  to 
precipitate  a  number  of  seizures.  Clark^  recommends  the  following 
as  an  emergency  prescription  when  status  is  threatened : 

I^ — Tr.  opii  deod.  lUv 

Potas.  bromid.  '  gr.  xxv 

Chlor.  hyd.  gr.  xx 

Liq.  morph.  sulph.  (U.  S.)  5J — M. 

S. — One  dose:  repeat  in  two  hours  if  necessary. 

This  prescription  may  be  given  after  the  first  four  or  five  seizures, 
after  that  sedation  must  be  pushed  to  control  the  attacks  as  they  are 
themselves  a  source  of  serious  danger  to  life.     Chloroform  may  be 

1  Wm.  P.  Spratling,  Epilepsy  and  its  Treatment,  Philadelphia,  1904. 


680         EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

given  in  emergency  by  inhalation,  but  the  most  valuable  of  the  drugs 
are  chloral  and  the  bromides.  While  it  is  necessary  to  push  them,  it 
must  be  constantly  kept  in  mind  that  these  very  drugs  are  contrain- 
dicated  in  the  next,  the  stuporous  stage,  and  therefore  no  more  should 
be  used  than  is  actually  necessary  to  control  the  situation  as  the  fol- 
lowing coma  will  be  deepened  thereby.  Chloral  and  bromides  are, 
as  a  rule,  best  given  by  rectum.  The  heart  needs  watching  and  may 
need  stimulating,  especially  if  large  doses  of  chloral  are  administered. 

In  the  stuporous  stage  the  treatment  is  stimulating  and  supporting. 
Careful  nursing  and  feeding  and  protection  during  the  great  exhaustion. 

Treatment  Between  Attacks. — ^As  there  is  no  disease  entity  (epilepsy) 
there  is  no  treatment  that  applies  to  all  of  the  cases  included  under 
that  term.  Each  of  the  various  conditions  requires  treatment  suited 
to  it  as  does  each  individual  require  individual  consideration. 

The  various  surgical  conditions,  tumor,  cyst,  abscess,  trauma,  etc., 
require  appropriate  surgical  intervention.  In  tumor,  for  example,  as  in 
other  organic  conditions,  when  the  location  of  the  trouble  is  not  evi- 
dent, a  study  of  the  attack  together  with  the  aura  may  give  valuable 
evidence  to  guide  the  surgeon.  It  is  desirable  to  have  all  such  cases 
reside  in  a  hospital  long  enough  for  their  attacks  to  be  accurately 
observed  before  operating. 

Conditions  of  infection  and  toxemia  require  no  special  mention  here. 
In  arteriosclerosis  with  softening  the  general  condition  overshadows 
the  special  manifestation  as  is  also  generally  the  case  following  hemor- 
rhage. Conditions  of  marked  arrest  of  development,  either  congenital, 
as  due  to  serious  birth  injuries,  or  early  inflammations  naturally  offer 
little  prospect  for  improvement. 

Internal  secretion  imbalances  should  be  corrected  as  far  as  possible, 
but  for  the  most  part  little  more  than  palliation  can  be  expected, 
although  the  near  future  may  well  have  something  to  offer  in  this  realm. 

Middle-ear  disease  should  be  adequately  treated  before  meningeal 
symptoms  and  lateral  sinus  thrombosis  take  place. 

The  syphilitic  meningitides,  whether  acquired  or  inherited,  offer  a 
promising  field  for  relief  by  appropriate  antisyphilitic  treatment, 
which  should  be  intensive  and  include  intravenous  injections  of 
salvarsan. 

As  to  the  pharmacotherapy  of  "  genuine  epilepsy"  it  can  be  inferred 
from  what  has  already  been  said  that  there  is  none  except  such  as  may 
be  called  upon  to  meet  or  prevent  emergencies  such  as  status.  Bro- 
mides have  been  so  constantly  used,  however,  that  they  need  to  be 
briefly  discussed. 

Bromide  acts  as  a  motor  depressant — it  raises  the  threshold  of 
motor  discharge  and  does  therefore  inhibit  the  convulsive  expression. 
That  it  does  this  is  sufficient  explanation  for  its  extensive  use.  Many 
popular  medicines  have  much  less  to  recommend  them.  When  we 
realize,  however,  that  the  convulsion  is  not  the  disease,  that  it  is  not 
the  cause  nor  a  first  expression  even  of  the  disease,  but  only  its  outward 
expression  and  the  end-result  at  that,  it  may  be  well  questioned  whether 


CLASSICAL  EPILEPSY  681 

bromide  medication  is  rational.  Experience  seems  to  show  that  the 
fit  postponed  by  bromides  comes  to  pass  ultimately  any  way  and  that 
the  bromide  may,  in  fact,  operate  unfavorably  by  tending  to  produce 
a  summation  of  attacks  and  thus  increase  the  danger  of  status.  Added 
to  this  is  the  potentiality  for  disturbing  digestion  which  the  bromides 
possess  so  prominently,  so  it  may  be  said  that  they  had  best  not  be 
given  at  all  unless  under  most  carefully  regulated  conditions.  The  true 
function  of  the  bromides  is  to  control  the  convulsive  manifestations 
when  they,  as  such,  become  a  source  of  danger  as  in  serial  attacks  and 
threatened  status. 

Bearing  in  mind  the  theory  of  the  disease  that  has  been  elaborated, 
the  rational  treatment  in  all  cases  where  the  underlying  mechanism 
cannot  be  unearthed  (as  in  the  symptomatic  epilepsies),  is  to  assist 
in  the  orderly  discharge  of  energy,  to  help  the  process  of  sublimation. 
This  is  best  effected  by  manual  training,  steady  occupation  graded  to 
suit  the  intellectual  level  and  other  requirements  of  the  patient,  and 
preferably  conducted  under  institutional  (colony)  supervision.  In 
many  individuals  much  is  to  be  expected  from  psychoanalysis.  No 
results  will  follow  from  short  treatment,  however.  The  most  favorable 
cases  need  from  twelve  to  eighteen  months. 

The  social  position  of  the  epileptic  is  most  pitiable,  often  so  impaired 
mentally  that  his  labor  is  at  a  discount,  he  loses  his  job  on  the  occa- 
sion of  the  first  fit,  even  though  he  may  have  succeeded  in  getting  one 
that  is  free  from  the  dangers  to  which  he  is  particularly  exposed  on 
account  of  his  infirmity.  He  is  thus  seriously  handicapped  in  the 
struggle  for  existence  and  barely  gets  to  earning  his  livelihood  in  one 
position  before  he  finds  himself  jobless  again,  without  recommenda- 
tion, and  forced  to  begin  all  over  again.  He  is  thus  apt  to  be  poorly 
nourished,  poorly  clothed,  and  the  subject  of  intense  social  repression 
operating  from  without  that  drives  him  back  upon  himself  and  aggra- 
vates greatly  his  trouble.  He  becomes  discouraged  and  depressed 
and  only  too  often  takes  to  alcohol,  the  very  worst  possible  thing  he 
could  do. 

In  the  colony  all  these  social  handicaps  are  removed.  Here  he  may 
have  a  fit  in  peace  and  comfort  without  feeling  that  he  is  disgraced  or 
in  imminent  danger  of  losing  his  means  of  livelihood.  This  relief  alone 
goes  a  long  way  toward  permitting  him  the  use  of  his  avenues  of  expres- 
sion and  in  bringing  about  a  relative  peace  and  quiet,  so  essential  as 
a  therapeutic  adjuvant.  If  in  addition  to  this  he  is  trained  in  some 
form  of  healthy  occupation,  preferably  outdoor,  that  is  interesting 
and  affords  an  added  means  of  expression  the  best  possible  has  been 
done  for  him.  In  the  colony,  too,  he  is  provided  a  home,  congenial 
surroundings,  a  regulated  diet,  and  is  under  that  careful  and  con- 
tinuous skilled  supervision  for  a  prolonged  time  which  is  so  essential 
to  the  best  results.  Under  colony  care  Spratling  thinks  5  per  cent, 
of  cases  as  they  go  can  be  cured  and  that  this  percentage  could 
be  doubled  or  perhaps  trebled  if  all  the  cases  could  be  gotten  under 
treatment  early. 


CHAPTER   XX. 
DEMENTIA  PRECOX  (SCHIZOPHRENIA)  GROUP. 

The  term  dementia  precox  has  been  the  occasion  of  a  great  deal 
of  discussion.  Coming  into  general  use  as  it  did  as  the  result  of  the 
studies  of  the  Kraepelinian  school  it  was  conceived  to  apply  to  a  group 
of  psychoses  belonging  to  the  period  of  adolescence  and  presenting 
dementia  as  a  fundamental  element  in  the  symptom  picture.  When, 
however,  it  was  seen  that  what  appeared  to  be  the  same  disease  might 
occur  later  in  life,  even  after  thirty  years  of  age,  it  seemed  hardly 
proper  to  use  the  term  precox  as  applied  to  psychoses  of  early  life. 
It  was  therefore  proposed  that  the  term  precox  should  refer  not  to 
the  age  of  the  patient,  but  to  the  relatively  early  appearance  of  demen- 
tia in  the  course  of  the  disease.  The  term  dementia  was  here  used 
to  mean  a  permanent  mental  impairment,  and  when  it  was  realized 
that  many  cases  made  good  recoveries  without  any  apparent  or  at 
least  material  defect  remaining,  another  reason  was  evident  for  the 
inapplicability  of  the  term.  The  concept,  then,  might  be  formulated 
that  it  was  a  disease  in  which  dementia  was  a  relatively  early  symp- 
tom, and  that  the  recoveries  occurred  only  when  the  disease  had  not 
progressed  to  any  extent.  This  also,  unfortunately,  does  not  meet 
the  facts,  because  many  cases  get  well  after  prolonged  and  apparently 
chronic  courses.  In  the  absence  of  any  well-defined  criteria  of  dementia 
it  was  impossible  to  predict  when  it  was  or  was  not  present,  and 
therefore  the  term  presents  very  many  undesirable  features.  Although 
it  is  somewhat  of  a  bootless  task  to  discuss  names,  and  although 
it  is  much  more  important  to  know  what  the  names  stand  for  than  to 
quibble  about  their  applicability,  still  it  is  of  course  desirable  to  have 
a  name  that  fairly  represents  the  thing  named.  To  meet  this  demand 
Bleuler  has  suggested  the  name  schizophrenia,  implying  a  splitting 
of  the  personality,  which  he  thinks  is  the  fundamental  symptom. 
Although  this  term  as  used  by  Bleuler  includes  a  number  of  conditions 
that  many  psychiatrists  would  object  to  as  being  included  in  the 
dementia  precox  concept,  still  it  is  generally  conceded  that  the  splitting 
of  the  personality,  as  indicated  by  the  name,  is  fundamental  in  this 
group,  and  the  name  is  coming  into  gradually  more  and  more  general 
use. 

Dementia  precox  must  undoubtedly  have  always  existed  and 
have  been  observed  by  physicians,  and  in  particular  the  grotesque 
cases  of  catatonic  rigidity  and  peculiar  mannerisms  must  have  always 
attracted  attention.     In  the  early  history  of  psychiatry,  however. 


ETIOLOGY  683 

few  descriptions  of  cases  exist  that  could  be  unequivocally  said  to  be 
cases  of  precox,  as  the  group  had  not  been  defined  from  other  groups 
superficially  resembling  it,  as  for  example,  imbecility.  Willis,  the 
English  anatomist,  recognized  as  early  as  1672  that  many  young 
people  underwent  deterioration,  and  Sydenham,  a  hundred  years 
later,  in  1772  describes  similar  conditions  under  the  description  of 
stupidity,  while  later  on,  after  mania  and  melancholia  had  been 
more  or  less  defined,  many  of  the  excitements  and  depressions  that 
are  incident  to  the  course  of  dementia  precox  were  undoubtedly 
grouped  under  these  headings,  while  at  one  period,  only  a  few  years 
ago,  there  was  a  distinct  group  supposedly  representing  a  special 
disease  described  by  the  name  of  catalepsy,  where  also  undoubtedly 
a  certain  number  of  precox  cases  were  arranged. 

At  the  present  time  three  pretty  well-defined  groups  of  cases  are 
included  in  the  general  concept  of  dementia  precox,  namely,  the  hebe- 
phrenic, the  catatonic,  and  the  paranoid.  Kahlbaum  was  the  first 
to  describe  hebephrenia  as  a  disease  entity  in  1863,  and  in  1871  his 
pupil,  Hecker,  published  some  excellent  descriptions  of  this  disease. 
In  1869  Kahlbaum  described  catatonia  under  the  term  Spannungsir- 
resein  or  vesania  catatonica,.  of  which  he  gave  an  admirable  mono- 
graphic description  in  1874.  In  1896  Kraepelin,  in  the  fifth  edition 
of  his  Lehrbuch,  arranged  dementia  precox,  catatonia,  and  dementia 
paranoides  as  disorders  of  metabolism.  Clouston,  the  Scotch  psychia- 
trist, had  already  described  what  he  termed  adolescent  insanity  and 
objected  to  the  term  dementia  precox  as  being  too  inclusive.  Kraepe- 
lin, however,  worked  over  his  material  with  great  thoroughness  and 
arrived  at  the  concept  that  includes  the  three  forms,  hebephrenic, 
catatonic,  and  paranoid  by  tracing  the  life  histories  of  his  patients 
and  grouping  all  these  cases,  however  dissimilar  they  might  appear 
on  the  surface,  from  the  standpoint  of  prognosis.  They- were  cases 
that  had  a  fairly  definite  course  and  outcome,  eventuating  always 
in  a  certain  degree  of  dementia. 

Etiology. — The  question  of  heredity  in  precox  has  been  studied, 
particularly  by  Wolfsohn,^  who  carefully  analyzed  the  material  from 
this  standpoint  at  the  Bergholzi  asylum  in  Zurich.  The  study  of 
2215  admissions  disclosed  647  cases  of  dementia  precox  of  whom 
90  per  cent,  showed  hereditary  taint.  Of  four  factors,  mental 
disease  was  the  most  frequent — about  64  per  cent. — followed  by 
nervous  diseases,  alcoholism,  and  other  forms  of  hereditary  taint. 
Heredity  was  combined  in  34  per  cent.  The  most  frequent  com- 
binations were  those  of  psychoses  and  alcoholism,  and  psychoses  and 
nervous  disease.  She  concluded  that  a  distinct  influence  of  heredity 
could  not  be  proved  in  the  cases  in  which  the  taint  was  alcoholism, 
nervous  disease,  or  other  forms.  The  catatonic  was  the  most  and 
paranoid  the  least  affected  by  the  mental  taint,  while  the  influence  of 

1  Die  Hereditat  bei  Dementia  Praecox,  Allg.  Zeitschr.  f.  Psych.,  1907,  Band.  Ixiv,  Heft 
2  and  3. 


684  DEMENTIA   PRECOX  GROUP 

the  taint  had  no  striking  effect  on  the  character  of  the  first  symptoms 
of  the  disease. 

It  would  seem  that  there  is  a  certain  element  of  direct  heredity, 
inasmuch  as  families  are  found  in  which  several  cases  of  precox  occur, 
just  as  there  are  families  found  in  which  several  cases  of  the  manic- 
depressive  psychosis  occur. 

Of  the  exciting  causes  severe  shocks,  both  mental  and  physical,  are 
not  infrequently  found,  as  for  example,  severe  hemorrhages  and  infec- 
tions following  parturition.  In  the  latter  case  precox  breakdowns 
occur  as  a  result  of  that  train  of  emotional  disturbances  which  follows 
upon  seduction  and  desertion. 

For  a  number  of  years  there  has  been  a  tendency  to  ascribe  the 
disease  to  disturbances  of  metabolism  with  possible  toxic  factors  and 
to  suppose  that  its  origin  might  be  traced  to  disorders  of  the  glandular 
secretions,  more  particularly  of  late,  of  the  internal  secretions  of  the 
ductless  glands,  and  inasmuch  as  the  disease  tends  to  focalize  about 
the  period  of  puberty  and  adolescence,  it  has  been  supposed  that 
perhaps  the  testicles  and  the  ovaries  might  be  the  offending  organs. 
This  whole  tendency  has  had  its  origin  largely  in  the  observed  physical 
changes  which  are  so  much  in  evidence  in  certain  cases.  More  will  be 
said  of  this  matter  later  when  the  discussion  of  the  nature  of  the  disease 
is  taken  up,  but  it  may  be  mentioned  here  that  whatever  its  ultimate 
nature  may  be  the  existence  of  toxic  factors  or  internal  secretory 
disturbances  is  purely  hypothetical  and  at  the  present  time  it  is 
more  useful  to  formulate  the.  upsetting  factors  as  well  as  the  general 
symptomatology  in  psychological  terms  rather  than  in  terms  descrip- 
tive of  disturbances  at  physicochemical  levels. 

The  formulation  of  the  disease  in  terms  of  the  affects  or  of  complexes 
or,  in  accordance  with  Meyer, ^  continued  unhealthy  biological  reac- 
tions, or  as  an  outgrowth  of  a  "shut  in"  character  is  after  all  more 
of  an  effort  of  description  of  what  is  found.  Everyone  has  complexes, 
but  it  is  not  clear  why  in  certain  cases  they  lead  to  the  development 
of  a  precox  psychosis,  while  a  "shut  in"  character  might  itself  in 
certain  cases  at  least  be  considered  to  be  an  early  expression  of  the 
disease  process,  a  latent  precox  perhaps,  in  the  sense  of  Bleuler. 

Symptoms. — Mental. — ^A  patient  from  time  to  time  writes  letters 
appealing  for  his  discharge  and  his  liberty  and  signing  himself 
"The  Emperor."  It  is  this  incongruity,  this  lack  of  oneness  of  the 
individual  that  for  a  long  time  has  attracted  attention  in  the  symp- 
tomatology of  this  disease.  How  is  it  possible  for  a  person  so  exalted 
as  to  think  himself  an  emperor  to  plead  in  quite  a  natural  way  for  his 
discharge  from  an  asylum?  The  two  positions  which  the  man  takes, 
acknowledging  himself  as  a  patient  and  pleading  for  his  discharge 
while  still  proclaiming  himself  an  emperor,  show  the  possibility  of 
maintaining  two  distinct  and  mutually  opposed  trends  of  thought 

1  Fundamental  Conceptions  of  Dementia  Praecox,  Brit.  Med.  Jour.,  September  29, 
1906. 


SYMPTOMS  685 

at  the  same  time  without  the  one  apparently  interfering  or  serving 
at  all  to  correct  or  modify  the  other. 

This  possibility  is  dependent  upon  a  fundamental  associational 
disturbance  which  has  caused  Bleuler  to  see  a  splitting  of  the  personality 
as  the  foundation  symptom  of  the  disease  and  to  give  it  the  name, 
therefore,  of  schizophrenia.  This  splitting  of  the  personality  has  been 
expressed  less  clearly  in  many  of  the  theories  that  have  been  advanced 
to  account  for  the  symptoms.  The  symptoms  have  been  said  to  be 
dependent  upon  a  disintegration  of  the  personality,  a  disintegration 
of  consciousness,  dissociation,  apperceptive  dementia,  and  the  weaken- 
ing of  consciousness  with  the  consequent  impairment  of  the  function 
of  the  real.  Reality  is  unable  to  correct  or  adequately  modify  the 
delusional  ideas,  with  the  result  that  such  bizarre,  strange,  and  ap- 
parently unps3"chological  modifications  of  conduct  are  seen. 

These  unpsychological  appearances  are  dependent  upon  what 
Bleuler  calls  autistic  thinking,  that  is,  a  form  of  thinking  to  which  Jung 
would  apply  the  term  introverted,  in  which  the  individual's  interests 
are  withdrawn  from  reality,  and  he  occupies  himself  with  himself 
to  the  more  or  less  complete  exclusion  of  the  outside  world.  This  is 
the  field  of  dream  formation,  of  phantasies,  wherein  things  come 
true.  An  analysis  of  cases  of  dementia  precox  shows  that  the  delusion 
formations  are  based  upon  wish-fulfilling  mechanisms  which  result 
oftentimes  in  highly  symbolic,  and  to  the  observer,  non-understandable 
expressions  which  are  formulated  in  accordance  with  the  particular 
complexes  which  may  be  operative.  These  mechanisms  are  the  same 
as  those  observed  in  normal  people,  in  hysterics,  and  those  suffering 
from  the  various  neuroses,  but  it  is  impossible  for  these  individuals 
to  adequately  utilize  them;  they,  therefore,  result  in  impairment  of 
efficiency  and  withdrawal  from  the  world  of  reality.  It  would  seem, 
too,  that  in  precox  the  regression  is  very  much  more  profound  than  in 
the  neuroses  and  the  psychoneuroses,  and  for  some  unknown  reason 
involves  a  serious  disintegration  of  the  personality  which  tends  to 
become  chronic  and  crippling.  From  the  standpoint  of  this  schizo- 
phrenic splitting  of  the  psyche,  based  upon  autistic  thinking,  many 
points  in  the  symptomatology  of  the  disease  become  understand- 
able. 

The  peculiar  emotional  dulness  and  uncertainty  of  emotional  re- 
sponse of  the  precox  has  long  been  noticed,  and  Stransky^  has  par- 
ticularly designated  it  by  the  term  of  intrapsychic  ataxia,  by  which 
term  he  means  a  disturbance  of  the  coordination  between  the  intellec- 
tual and  the  affective  attributes  of  the  psyche,  which  are  respectively 
known  as  the  noopsyche  and  the  thymopsyche.  This  noo-thymopsy- 
chic  ataxia  gives  the  appearance  at  times  of  emotional  dulling  and 
at  other  times  of  a  senseless  emotional  reaction.    Thus  a  patient  who 

1  Ueber  die  Dementia  Prsecox,  Streifziige  durch  Klinik  und  Psychopathologie,  Verlag. 
von  T.  F.  Bergmann,  Wiesbaden,  1909. 


686  DEMENTIA   PRECOX  GROUP 

receives  news  of  the  death  of  a  near  relative  unmoved,  may  a  Httle 
later  on  laugh  heartily  at  apparently  nothing.  He  is  happy  when  he 
should  be  sad,  sad  when  he  should  be  happy,  angry  without  cause, 
experiencing  fear  without  reason,  for  the  most  part  cold  and  impas- 
sive, but  occasionally  showing  outbursts  of  marked  and  accentuated 
emotionalism.  This  is  the  "April  weather"  behavior  of  the  affects, 
in  the  language  of  Stransky. 

This  condition  of  affairs  is  understandable  upon  the  basis  of  the 
splitting  of  the  psyche.  The  emotional  reactions  occur  when  the 
complexes  have  been  touched,  which  the  individual  is  constantly  in 
an  attitude  of  trying,  to  prevent.  This  method  of  dealing  with  the 
emotions  is  well  known,  particularly  in  the  formation  of  the  dream, 
in  which  the  mechanism  of  displacement,  by  removing  the  affect  from 
the  constellation  of  ideas  to  which  it  belongs  and  attaching  it  to  an 
indifferent  set  of  ideas  serves  to  hide  from  the  patient  the  realization 
of  the  actual  difficulty.  For  example,  a  patient  identifies  herself  with 
a  schoolmate  of  her's  and  then  accuses  the  schoolmate  of  being  "bad" 
and  pregnant.  It  will  be  easilj^  seen  that  in  such  a  case  the  patient 
is  protecting  herself  from  the  realization  that  she  has  thoughts  to 
which  the  term  "bad"  might  be  applied,  and  that  the  pregnancy  which 
might  result  from  being  bad  is  transferred  to  her  schoolmate.  Under 
such  circumstances,  she,  so  to  speak,  unloads  her  emotion  upon  this 
schoolmate  and  very  easily  may  produce  the  impression  of  indifference 
toward  herself  with  an  unmotived  affective  attitude  toward  the 
schoolmate. 

This  withdrawal  from  reality,  this  looking  within,  occupying  them- 
selves with  themselves,  no  longer  subject  to  the  corrective  infiuences 
of  the  outside  world,  produces  many  surface  indications,  among  which 
axe  failures  of  voluntary  attention,  lack  of  interest,  disturbances  of  orienta- 
tion, disorders  of  memory.  The  disorders  of  attention,  lack  of  interest, 
failure  of  voluntary  attention  can  easily  be  seen  to  be  due  to  the 
turning  of  the  interests  within.  The  capacity  for  attention  may  be 
as  keen  as  ever,  but  the  patients  are  not  attending  to  the  things  going 
on  about  them,  but  rather  to  the  things  going  on  within,  and  so  they 
apparently  take  no  interest  in  the  people  or  the  events  of  their  environ- 
ment. They  may  even  express  themselves  as  perfectly  satisfied  with 
their  confinement  in  a  hospital,  and  be  so  manifestly  heedless  of 
those  about  them  that  it  is  practically  impossible  to  draw  them  into 
conversation.  This  lack  of  interest  and  attention  naturally  produce 
what  appear  to  be  disturbances  of  memory  and  orientation.  The 
patient,  who  is  heedless  of  his  surroundings,  may  easily  not  know  the 
day  of  the  week  or  may  have  forgotten  the  events  that  only  recently 
took  place  about  him,  because  they  were  not  sufficiently  attended  to 
to  make  any  profound  impression,  while  a  patient  who  identifies  him- 
self with  some  great  public  functionary  might  easily  not  give  the 
correct  date  of  his  own  birth.  Such  considerations  as  these  show  how 
necessary  it  is  to  penetrate  beneath  the  surface  indications  which 


SYMPTOMS  687 

the  patient  manifests  and  find  out  their  true  meaning.  They  also 
indicate  unequivocally  that  what  may  appear  quite  unpsychological 
is  really  perfectly  understandable. 

The  same  type  of  explanation  serves  to  render  clear  the  meaning 
of  such  surface  indications  as  the  shallowness  of  thinking  and  the 
apparent  dilajndation  of  thought.  The  interests  and  the  energies  are 
occupied  with  things  which  are  not  accessible  to  casual  questioning. 
In  fact,  as  shall  later  on  be  seen,  the  patient  may  be  wholly  inaccessible 
to  any  form  of  approach,  and  when  there  is  considerable  speech  produc- 
tivity what  is  said  may  be  so  incoherent  upon  the  surface  as  to  be 
quite  non-understandable  and  constitute  what  has  been  called  a 
"word  salad." 

The  delusional  formation  of  precox  is  notoriously  grotesque  and 
partakes  of  this  characteristic  to  such  an  extent  in  harmony  with 
the  grotesqueness  of  the  thoughts  in  dreams  that  the  similarity  has 
not  been  overlooked.  The  mechanisms,  too,  are  probably  quite  the 
same,  although  much  more  difficult  to  fathom  because  of  the  inaccessi- 
bility and  lack  of  cooperation  of  the  patient.  If  the  eye  is  properly 
trained,  however,  to  see  meaning  in  the  apparently  meaningless, 
there  will  be  little  difficulty  in  seeing  how  certain  expressions  may 
be  interpreted,  even  though  in  an  individual  case  it  may  be  impossible 
to  verify  such  an  interpretation.  For  example,  an  old  precox  who 
talked  in  a  thoroughly  dilapidated  manner  but  was  able  with  some 
patience  to  give  a  fairly  good  account  of  himself,  injected  into  his 
series  of  replies  to  questions  that  the  President  was  confined  in  an 
almshouse  and  that  he  had  come  to  Washington  to  be  President. 
He  also  stated  that  he  had  had  something  to  do  with  his  sister  when 
he  was  a  young  boy,  that  he  did  not  tell  the  priest  and  that  his  shadow 
was  very  heavy,  that  it  was  black,  and  that  he  saw  the  Devil  in  it. 
Here  an  expression  of  grandiose  ideas,  which  speaking  generally  may 
be  considered  as  compensatory  are  seen.  In  his  autistic  thinking  he 
wishes  to  be  the  great  man  that  in  his  real  life  he  is  not.  Then  one 
sees  the  possibility  of  a  serious  moral  confiict,  the  result  of  incestuous 
relations  with,  or  perhaps  only  incestuous  thoughts  about,  his  sister, 
while  his  dark  and  heavy  shadow  in  which  he  sees  the  Devil  can  be 
easily  seen  to  be  a  symbolic  representation  of  the  destructive  effects 
which  his  moral  delinquencies  have  had  upon  him.  Here  one  also 
sees  that  the  hallucinatory  experiences,  the  disorders  of  the  sensorium 
express  themselves  as  symbolisms  of  the  conflict  and  receive  their  inter- 
pretation with  a  knowledge  of  the  nature  of  that  conflict. 

The  delusions  are  essentially  endogenous  in  origin,  that  is,  ulti- 
mately dependent  upon  factors  that  are  within  the  individual,  and 
they  tend  to  be  colored  and  determined  by  complexes  which  lie  at  the 
very  foundation  of  the  personality,  which  have  to  do  with  the  region 
of  the  psyche  which  has  long  since  been  forgotten  and  to  which  Freud 
gives  the  name  "unconscious."  It  is  largely  because  of  their  pro- 
foundly unconscious  origin  that  they  are  inaccessible  and  it  is  largely 


DEMENTIA   PRECOX  GROUP 

owing  to  this  also  that  they  produce  such  serious  disturbances  and 
such  marked  departures  from  conduct  that  is  at  all  efficient. 

Just  as  the  hallucinations  are  symbolisms  of  the  elements  in  the 
conflict  so  are  the  various  mannerisms,  stereotypies,  and  neologisms. 
All  of  these  motor  disturbances  are  in  effect  symptom  activities,  complex 
indicators,  and  serve  in  some  way  to  portray  the  nature  of  the  conflict. 
In  a  recently  reported  case,  for  example,  an  old  precox  was  observed 
to  keep  pounding  one  hand  with  her  clenched  fist  in  a  rhythmic  stereo- 
typed fashion.  It  was  discovered  that  in  her  earlier  days  she  had 
been  jilted  by  a  shoemaker.  This  peculiar  action  could  be  seen,  in 
the  light  of  this  knowledge,  as  but  the.  movements  of  the  shoemaker 
pounding  at  his  last.  Many  of  the  old  cases  of  precox  have  such 
stereotyped  activities  which  it  is  often,  in  fact  usually,  quite  impossible 
to  fathom,  but  in  the  light  of  such  a  case  as  this  it  will  be  seen  that 
they  must  all  be  looked  upon  as  having  meaning  and  that  for  a  com- 
plete reconstruction  of  the  psychosis  it  is  necessary  to  determine  what 
that  meaning  is. 

As  an  example  of  what  painstaking  analysis  may  disclose  may  be 
mentioned  a  patient  of  Jung's.  She  expressed  herself  in  stereotyped 
and  apparently  meaningless  phrases  interspersed  with  neologisms.  One 
of  her  statements  was:  "I  affirm  a  million  Hufeland  to  the  left  on 
the  last  fragment  of  earth  on  the  hill  above."  A  detailed  analysis 
disclosed  this  sentence  to  mean,  approximately:  "For  the  bad  treat- 
ment of  the  physicians  which  I  have  to  endure  here  and  with  which 
I  am  tortured  to  death  I  claim  a  high  indemnity." 

Negativism,  one  of  the  characteristic  symptoms  of  precox,  may  be 
expressed  passively  by  the  patient's  not  doing  what  is  expected  of 
him,  or  what  he  should  do,  or  by  actively  doing  the  exact  opposite  of 
what  is  requested.  The  passive  negativism  may  show  itself  in  a 
refusal  to  attend  to  the  promptings  of  normal  desires,  so  that  the 
bladder  and  the  rectum  are  permitted  to  become  overloaded  and  the 
saliva  to  collect  in  large  quantities  in  the  mouth,  or,  on  the  other 
hand,  active  negativism  may  show  itself  in  the  patient  doing  the 
exact  opposite  of  what  he  is  asked  to  do,  for  example,  if  he  is  asked 
to  shut  his  eyes  he  opens  them  wider  and  if  he  is  asked  to  open  them 
he  will  shut  them  tightly,  and  if  he  is  asked  to  put  out  the  tongue  he 
shuts  his  lips  tightly,  and  if  he  is  asked  to  shut  his  mouth  he  opens 
it,  etc.  This  peculiar  symptom  is  dependent  upon  what  Bleuler  terms 
the  amhivalency  of  ideas,  by  which  he  means  that  every  idea  has  con- 
nected with  it  by  association  its  exact  opposite  more  intimately  than 
other  ideas,  and  so  the  patient  who  is  withdrawn  from  reality  and 
objects  to  being  invaded  by  the  world  of  real  things,  who  would,  in 
other  words,  live  within  himself,  finds  that  in  refusing  to  accord  with 
a  suggestion  from  the  outer  world  the  path  of  the  diametrically  oppo- 
site reaction,  is  more  patent,  more  accessible  than  any  other.  This 
is  a  type  of  reaction  which  one  sees  also  in  other  conditions,  as  in 
hysteria,  and  also  quite  naturally  in  children. 


SYMPTOMS  689 

The  suggestibility  of  the  precox  receives  a  similar  explanation. 
Some  patients  find  it  easier  to  follow  blindly  any  suggestion  which 
comes  to  them  from  any  source  than  to  actively  initiate  contact  with 
reality.  One  precox  patient,  for  example,  had  to  be  fed  by  placing  his 
food  before  him  and  a  spoon  in  his  hand  and  repeating  each  time  the 
command  to  take  another  mouthful.  After  having  responded  to  the 
command  he  remained  inert  until  it  was  repeated.  This  form  of 
activity  requires  no  initiative  on  the  part  of  the  patient,  no  actual 
effective  contact  with  reality  of  his  own  devising  and  permits  him  to 
remain  within  himself,  much  as  does  the  negativistic  tendency. 

The  catatonic  rigidity  of  the  precox  is  a  still  more  active  shutting 
out  of  the  world  to  the  point  of  absolute  inattention  to  the  environ- 
ment, but  an  inattention  which  is  positive  and  active  rather  than 
passive,  as  in  catalepsy  and  command  automatism,  while  stuyor  still 
more  effectively  shuts  out  the  world  of  real  things. 

There  is  a  reaction  type  in  dementia  precox  which  is  important  for 
an  understanding  of  the  nature  of  the  disease.  It  is  the  archaic  type 
of  reaction .  In  the  illustrations  that  have  been  given  it  is  seen  that 
when  the  individual  under  the  influence  of  mental  disease  regresses  he 
not  only  reaches  lower  levels,  but  frequently  reaches  levels  corre- 
sponding with  his  early  infancy,  and  so  it  is  frequently  found  that  the 
delusions  and  other  morbid  manifestations  only  find  their  explanation 
when  traced  back  to  the  infantile  period.  In  the  same  way  regressions 
may  lead  back  to  lower  cultural  levels  so  that  patients  show  symptoms 
that  are  only  understandable  in  terms  of  the  psychology  of  more 
primitive  peoples.  The  materials  of  experience  are  used  by  the 
patient  in  an  archaic  way.  One  such  patient  with  a  very  complicated 
delusional  system  states  that  he  is  the  father  of  Adam,  that  he  has 
lived  in  his  present  human  body  thirty-five  years,  but  in  other  bodies 
thirty  million  years,  and  that  during  this  time  he  has  occupied  six 
million  different  bodies.  He  has  been  the  great  men  in  the  history 
of  the  development  of  the  human  race;  he  himself  created  the  human 
race;  it  took  him  three  hundred  million  years  to  perfect  the  first 
fully  developed  human  being;  he  is  both  male  and  female,  and  identi- 
fies all  the  different  parts  of  the  universe  with  his  own  body;  Heaven, 
Hell  and  Purgatory  are  located  in  his  limbs,  the  stars  are  pieces  of 
his  body  which  have  been  torn  apart  by  torture  and  persecution  in 
various  ages  of  past  history;  he  is  the  father  and  creator  of  the  various 
races  and  elements  of  the  human  organization,  etc.  Here  is  a  very 
primitive  type  of  thinking  in  which  the  patient  identifies  himself  with 
the  whole  universe  somewhat  as  the  baby  does  and  somewhat  as 
primitive  man  does.  He  is  quite  in  the  position  of  the  chief  of  some 
primitive  tribes  in  whom  all  the  forces  of  the  universe  center  and 
from  whom  radiate  all  of  these  forces  for  good  or  for  ill.  This  is  the 
archaic  type  of  reaction  which  shows  how  deep  the  split  of  the  person- 
ality may  be,  how  fundamental  it  may  be,  and  gives  an  insight  into 
the  seriousness  of  the  disease  process. 
44 


690  DEMENTIA   PRECOX  GROUP 

Physical. — A  considerable  number  of  precox  patients,  particularly 
the  catatonic  and  the  hebephrenic,  show  marked  physical  symptoms, 
and  not  infrequently  have  all  the  outward  appearances  of  being  quite 
ill.  They  often  emaciate  during  the  early  period  of  their  illness,  suffer 
from  anorexia  and  insomnia,  circulatory  disturbances,  disturbed  car- 
diac action,  cyanosis  of  the  extremities,  vasomotor  disorders  of  which 
dermographia  is  not  an  infrequent  manifestation.  The  deep  reflexes 
are  commonly  exaggerated,  while  the  pupils  in  this  class  of  cases  are 
characteristically  widely  dilated.  Convulsive  seizures  of  an  epilepti- 
form, but  more  often  of  an  hysteriform,  variety  may  occur. 

In  the  very  early  stages  of  the  disease  physical  symptoms  which 
do  not  lead  to  the  suspicion  of  mental  disturbance  are  not  infrequent. 
Such  symptoms  as  headache  may  be  in  evidence  for  a  considerable 
time  as  may  also  vertigo,  and  Urstein^  has  called  especial  attention 
to  the  occurrence  of  gastric  disturbances.  Other  physical  disturbances 
may  also  of  course  occur,  and  if  no  adequate  foundation  can  be  found 
for  them  a  mental  origin  should  be  thought  of. 

It  is  this  type  of  physical  illness  that  has  led  to  and  maintained  the 
belief  in  etiological  factors  at  the  biochemical  level. 

Mode  of  Onset.^ — The  early  manifestations  of  precox  vary  greatly. 
As  already  indicated  the  disease  may  remain  latent  for  a  considerable 
period,  manifesting  itself  only  in  slight  disturbances,  predominantly 
of  a  physical  nature,  such  as  headaches  and  gastro-intestinal  disorders. 
In  quite  a  large  percentage  of  cases  a  "shut-in"  type  of  character  has 
been  found  to  have  existed  for  a  considerable  period  before  the  out- 
break of  the  symptoms,  in  fact  to  have  been  a  characteristic  of  the 
individual  before  the  breakdown.  Whether  this  is  to  be  considered 
as  a  symptom  of  the  disorder  or  an  expression  of  the  type  of  individual 
in  whom  the  disorder  is  possible  cannot  be  answered  at  this  time. 

In  sharp  contrast  to  these  latent  periods  the  disorder  develops 
not  infrequently  with  great  suddenness.  After  some  emotional  shock 
the  patient  becomes  almost  immediately  greatly  confused  or  catatonic. 

It  is  quite  frequent  to  have  the  break-down  be  a  slowly  progressive 
developing  condition.  During  the  early  period  the  symptoms  may 
take  all  sorts  of  forms  and  may  easily  be  mistaken  for  types  of  manic- 
depressive  psychosis,  compulsion  neuroses,  neurasthenia,  hysteria, 
hypochondria,  acute  confusion  and  paranoid  states.  Anything 
atypical  in  these  psychoses  should  make  one  think  of  the  possibility 
of  dementia  precox. 

The  varieties  of  dementia  precox  will  be  described  under  five  heads : 
I.  Dementia  Simplex;  II.  Hebephrenia;  III.  Catatonia;  IV.  Para- 
noid Forms;  V.  Mixed  and  Atypical  Forms. 

I.  Dementia  Simplex. — In  this  group  of  cases  the  origin  is  usually 
insidious,  perhaps  manifesting  itself  only  by  slight  physical  disturb- 
ances, such  as   headache,  gastro-intestinal    attacks,  by   some    irri- 

1  Die  Dementia  Praecox  und  Ihre  Stellung  zum  manisch-depressiven  Irresein,  Berlin 
U.  Wien,  1909. 


DEMENTIA  SIMPLEX  691 

tability,  and  perhaps  a  tendency  to  withdraw  from  the  association  of 
others.  The  patient  may  suffer  from  insomnia,  perhaps  disagreeable 
dreams,  and  there  may  be  passing  evidences  of  hallucinations  and 
delusions.  These  are  apt  to  be  expressed  only  at  times,  perhaps 
under  the  influence  of  a  little  excitement,  and  show  little  tendency  to 
organization  or  progression. 

The  patient  emotionally  is  more  apt  to  be  somewhat  depressed,  or 
at  least  indifferent,  lacking  in  initiative,  and  presenting  outwardly  an 
appearance  that  frequently  leads  to  the  diagnosis  of  a  "  nervous  break- 
down," "nervous  prostration,"  or  neurasthenia. 

Such  mild  attacks  as  this  may  be  pretty  well  recovered  from  but 
may  recur,  and  one  not  infrequently  finds  a  history  of  one  or  more 
such  attacks  having  preceded  a  more  severe  break-down. 

It  is  this  group  of  cases  of  mild  abortive  forms,  "formes  fntstes" 
of  the  French,  which  after  recovery  from  a  "flurry"  or  "excited 
episode,"  gives  one  the  impression  of  "peculiar  characters."  Many 
such  cases  are  found  in  the  ranks  of  the  criminals,  hoboes,  prostitutes, 
pseudogeniuses,  cranks,  and  eccentrics,  and  accurate  analyses  of 
the  histories  in  these  cases  would  not  infrequently  show  a  precox 
episode  which  separated  a  period  of  relative  efficiency  in  their  lives 
from  the  period  following  relative  inefiiciency,  in  which  latter  it 
might  be  possible  to  detect  certain  precox  residuals. 

An  analysis  of  the  life  histories  of  this  class  of  cases  shows  quite 
regularly  a  constant  tendency  to  slip  from  under  all  forms  of  responsi- 
bility, and  a  lack  of  capacity  for  any  kind  of  continuous  application 
and  inability  to  develop  the  habit  of  work.  They  characteristically 
resort  to  the  hobo  type  of  existence,  are  unable  to  adapt,  with  any 
degree  of  efficiency  at  all,  complex  conditions  of  life,  and  wander 
from  place  to  place,  occupying  one  position  after  another  from  which 
they  are  either  dismissed  because  of  inefficiency  or  leave  voluntarily, 
giving  reasons  for  so  doing  which  are  totally  inadequate.  Such  cases 
as  these,  sometimes  by  a  steady  progress  become  very  greatly  dilapi- 
dated, and  it  is  quite  surprising  at  times  to  find  the  amount  of  deteriora- 
tion in  such  cases  after  their  admission  to  an  institution  and  to  realize 
how  long  they  have  gotten  on  in  the  outer  world  in  a  seriously  mentally 
crippled  condition.  Of  course  their  continuance  in  the  outer  world 
was  made  possible  only  by  their  having  sunk  to  low  and  relatively 
simple  social  levels  that  made  little  or  no  demands  upon  them. 

When  such  patients  as  these  get  into  situations  which  require 
continuity  of  effort  and  constant  adjustment  and  from  which  they 
cannot  escape  they  not  infrequently  suffer  from  severe  and  more 
acute  breaks.  This  is  seen  in  the  military  service.  The  Army  and 
the  Navy  naturally  attract  this  wandering  horde  of  inefficients  who 
see  in  the  mihtary  service  only  the  glitter  of  brass  buttons  and  the 
opportunity  to  see  the  world.  After  enlistment,  however,  when  they 
are  required  to  take  up  the  grind  of  daily  work,  their  defects  soon 
come  into  the  foreground  and  manifest  themselves  either  by  distinct 


692  DEMENTIA   PRECOX  GROUP 

psychotic  episodes  or  by  minor  infractions  of  military  discipline, 
such  as  staying  away  from  the  post  beyond  the  leave  granted,  or  per- 
haps more  serious  offences,  such  as  desertion.  Such  cases  when  finally 
they  come  under  observation  in  a  hospital  easily  show  their  defect. 

Many  women  of  this  type  marry,  have  children  and  although 
looked  upon  as  "unique,"  or  "queer,"  nevertheless  get  along  if  there 
is  no  economic  strain. 

II.  Hebephrenia. — This  form  of  dementia  precox  is  more  severe 
than  the  preceding.  It,  however,  is  not  essentially  different,  being 
practically  the  same  condition  occurring  with  greater  severity,  more 
apt  to  be  somewhat  more  acute  in  its  onset,  and  manifesting  itself 
more  prominently  by  hallucinations  and  delusions. 

The  onset  of  hebephrenia  not  infrequently  presents  the  outward 
appearances  of  a  depression  and  so  resembles  the  depressive  phase  of 
a  manic-depressive  psychosis,  or  perhaps  some  other  form  of  depres- 
sion. It  not  infrequently  happens,  on  the  other  hand,  that  the  first 
appearances  of  the  disorder  are  those  rather  of  mental  confusion  than 
of  a  marked  depression.  These  early  manifestations  are  the  method 
of  reaction  of  the  individual  to  the  first  outbreak  of  the  conflict.  He 
may  become  either  greatly  depressed  or  may  be  quite  unable  to  react 
along  any  well-defined  line  and  become  quite  confused.  From  this 
acute  condition,  after  a  longer  or  shorter  duration,  he  settles  down 
into  a  more  stereotyped  expression  of  psychotic  symptoms. 

During  these  early  stages  the  productivity  is  not  infrequently 
deliroid  in  character,  accusing  voices  are  heard  and  rather  ill-defined 
and  not  well-formulated  delusions  are  expressed  which  are  characteris- 
tically of  a  self-accusatory  type  and  in  harmony  with  the  depression. 
Attempts  at  suicide  not  infrequently  occur  during  this  period. 

After  the  active  symptoms  of  the  onset  have  subsided  there  is  a 
settling  down  into  a  more  stereotyped  delusional  expression,  but  not 
infrequently  with  a  more  or  less  incoherent  productivity  and  with 
expressions  that  sound  fantastic  and  silly.  One  patient  complains 
that  the  sheets  stick  to  his  feet,  another  than  he  is  the  "wandering 
planet."  Such  ideas  appear  to  have  no  adequate  reason  and  are 
expressed  quite  disconnectedly  from  the  general  train  of  thought  and 
little  or  no  attempt  is  made  to  support  them  by  logic.  One  patient, 
for  example,  says  that  his  enemies  are  following  him,  and  that  he  has 
been  killed  a  number  of  times.  Another  complains  that  other  patients 
are  trying  to  injure  him.  All  of  these  statements  are  made  without 
show  of  emotion  in  a  decidedly  matter-of-fact  way.  Such  appear- 
ances as  these  have  led  to  such  descriptive  phrases  as  looseness  of  the 
train  of  thought,  poverty  of  ideas,  emotional  deterioration.  From  the 
previous  discussion,  however,  of  the  meaning  of  symptoms  it  is  known 
that  the  hallucinations  are  expressions  of  the  conflict,  that  the  delu- 
sions are  compromise  formations,  and  that  the  apparent  incoherency 
of  the  speech  does  not  necessarily  imply  an  incoherency  in  the  thought 
content.    It  is  usually  not  especially  difficult  to  find  some  fairly  direct 


HEBEPHRENIA  693 

connection  between  the  accusations  of  the  voices  and  actual  occur- 
rences in  the  patient's  life.  Similarly  with  other  disorders  of  the 
sensorium.  One  patient  for  example,  who  had  seduced  a  girl,  who 
bore  him  an  illegitimate  child,  saw  visions  of  his  mother  and  heard 
the  young  woman's  voice  telling  him  to  come  home,  to  go  to  work,  and 
lead  a  decent  life. 

The  peculiar  emotional  reaction,  the  lack  of  interest,  and  apparent 
emotional  dilapidation,  as  noted,  is  due  to  displacement,  and  so 
it  is  perfectly  understandable  that  when  an  effort  is  made  to  gain 
access  to  such  individuals  that  they  show  little  interest  and  character- 
istically reply  to  questions  addressed  to  discovering  the  reasons  for 
their  actions  by  "I  don't  know."  It  is  really  quite  true  that  they 
do  not  know  the  reasons  for  their  actions  any  more  than  any  person 
understands  the  reasons  for  the  different  appearances  that  have  come 
to  him  in  a  dream.  And  then  again  these  patients  are  so  occupied 
with  themselves  that  they  do  not  care  often  to  be  interfered  with,  and 
the  "I  don't  know"  is  as  much  as  to  say  "leave  me  alone." 

The  general  conduct  of  the  patient  may  also  be  listless,  apathetic 
and  disinterested  in  character  and  in  harmony  with  his  emotional 
condition,  whereas  mild  alternations  of  depression  and  of  excitement 
with  the  development  of  little  peculiarities  of  conduct  and  speech 
show  the  alliance  between  this  group  of  cases  and  the  catatonics. 
One  patient,  for  example,  who  thought  that  he  was  ordained  to  preach 
and  that  the  bread  was  impure,  would  be  quiet  for  months,  and  then 
exhibit  a  certain  degree  of  restlessness  by  following  the  doctors  and 
nurses  about  the  wards  telling  them  his  troubles  for  a  few  days.  The 
alternation  may  be  much  more  marked  as  between  a  state  of  stupor 
and  a  state  of  excitement  in  which  the  patient  eats  paper,  strings, 
and  bedecks  himself  with  all  manner  of  trash  and  shows  outbreaks  of 
violent  anger.     This  condition  approaches  much  closer  to  catatonia. 

Many  of  the  cases,  however,  do  not  show  that  amount  of  dilapida- 
tion which  the  previous  description  would  imply,  while  any  degree 
of  incoherence  may  be  seen  up  to  the  production  of  a  veritable  "  word 
salad."  On  the  other  hand  the  patients  may  be  found  well  oriented, 
making  outwardly  a  natural  appearance,  being  able  to  give  a  very 
good  account  of  themselves,  but  presenting  a  grotesque  delusional 
system,  supported  by  hallucinations  the  expression  of  which  stands 
in  rather  striking  contrast  to  the  outward  appearances.  These 
delusional  systems  represent  all  degrees  of  coherence  and  it  would 
seem  that  the  ability  on  the  part  of  the  patient  to  formulate  a  coherent 
and  consistent  delusional  world  was  one  expression  at  least  of  his 
capacity  to  react  to  the  destructive  influences  of  the  conflict. 

One  such  patient  complained  of  illness  on  a  train  and  told  the 
conductor  that  she  had  had  a  hemorrhage.  No  sign  of  any  hemor- 
rhage was  in  evidence,  however,  and  when  she  reached  the  sanitarium 
to  which  she  was  removed  nothing  abnormal  was  found  on  examina- 
tion.    When  her  mother  arrived  shortly  afterward  she  was  found 


694 


DEMENTIA   PRECOX  GROUP 


delirious.  Later,  upon  her  admission  to  a  hospital  she  was  very 
hypochondriacal,  talked  at  length,  complained  of  attacks  of  hemor- 
rhage from  the  vagina,  which  hemorrhage  she  said  was  due  to  an 
assault  while  she  slept  either  by  a  man  or  some  sharp  instrument. 
Later  on  she  claimed  to  be  married  to  a  Mr.  O.  and  wrote  long  letters 
giving  an  account  of  her  troubles  and  giving  an  account  of  the  hemor- 
rhages and  the  supposed  operation,  showing  an  extensive  delusional 
system.  She  claimed  to  have  been  the  victim  of  a  criminal  operation 
performed  by  a  doctor.  She  later  said  that  she  had  been  engaged  to 
a  Hebrew  before  she  was  born  and  that  at  twenty  she  broke  this 
engagement,  that  at  three  years  of  age  a  Catholic  priest  requested  one 
of  her  offspring  be  given  to  that  church  for  a  clergyman  and  that 
the  Hebrew  family  consented.  In  the  midst  of  a  great  mass  of  inco- 
herent jumbled  delusional  expressions  evidences  of  a  compensatory 


Fig.  307. — Appearance  of  patient  in  catatonic  stupor. 


wish-fulfil ing  delusional  system  are  found.  Wishing  for  a  child  she 
became  impregnated.  Being  a  virtuous  woman  this  has  to  be  accounted 
for.  She  therefore  has  the  delusion  that  she  is  married  to  Mr.  O. 
Inasmuch  as  no  child  appears  a  delusion  that  she  has  had  an  abortion 
performed  accounts  for  its  absence,  and  as  this  is  a  criminal  operation 
it  was  performed  without  her  knowledge  while  she  slept.  These  delu- 
sions are  mixed  up  with  all  sorts  of  ideas  about  the  dislocation  of  her 
various  viscera,  numerous  hemorrhages  from  the  vagina,  and  a  frac- 
tured skull,  etc.  It  is  loosely  organized  and  interrupted  by  violent 
outbreaks,  restlessness  and  irritability. 

She  has  been  unable  to  adjust  to  reality,  the  effort  at  compensation 
by  delusional  formation  has  also  failed,  with  the  resulting  progressive 
dilapidation  of  coherence  in  the  stream  of  thought. 

III.  Catatonia. — This  variety  of  precox,  like  the  hebephrenic,  may 
come  on  suddenly  with  symptoms  of  confusion  or  depression,  or  may 
be  of  somewhat  slower  onset.  It  is  more  apt  than  the  other  forms  to 
be  of  relatively  acute  onset,  in  which  case  it  sometimes  follows  a  sud- 
den shock  of  a  highly  emotional  character.    It  is  characterized  more 


CATATONIA 


695 


especially  by  a  predominance  of  motility  disturbances  and  tends  to 
express  itself  in  alternating  conditions  of  catatonic  stupor  and  catatonic 
excitement. 

In  the  conditions  of  catatonic  stupor  negativism  reaches  a  very  high 
degree.  The  patients  are  perfectly  immobile,  sitting  off  in  corners  by 
themselves  or  lying  in  bed  without  paying  any  apparent  attention  to 
what  goes  on  about  them,  are  quite  inaccessible,  fail  to  answer  ques- 


FiG.  308. — Catalepsy;  flexibilitas  cerea. 


tions,  and  do  not  react  at  all  to  stimulii  from  the  outer  world.  They 
characteristically  often  refuse  to  speak  at  all.  This  mutism  is  a 
manifestation  of  the  negativism.  Besides  this  the  patients  often 
refuse  food,  pay  no  attention  to  the  promptings  of  the  bladder  and 
the  rectum  which  become  overloaded .  with  urine  and  fecal  matter, 
often  to  a  serious  extent.  Saliva  may  be  permitted  to  accumulate  in 
the  mouth  where,  if  attention  is  not  paid  to  it,  it  may  undergo  putre- 
factive changes.    The  patients  quite  characteristically  show  peculiar 


696  DEMENTIA   PRECOX  GROUP 

theatrical  attitudinizing,  make  grimaces,  occupy  peculiar  positions, 
and  if  they  speak  the  productivity  is  often  incoherent  and  apparently 
senseless,  with  a  tendency  to  constant  repetition  of  the  same  phrases 
— perseveration — which  may  also  manifest  itself  in  the  movements  of 
the  body,  such  as  a  constant  swaying  movement  or  the  like. 

The  muscular  system  may  be  in  a  condition  of  waxy  flexibility, 
permitting  of  the  molding  of  the  limbs  into  any  position  where  they 
remain  indefinitely — catalepsy.  When  this  is  present  the  patients 
tend  to  show  a  more  or  less  high  degree  of  suggestibility  and  command 
automatism,  doing  mechanically  and  in  a  perfectly  automatic  manner 
that  which  they  are  commanded  to  do.  This  suggestibility  shows 
itself  further  in  echolalia,  the  repetition  of  words  or  phrases  that 
are  addressed  to  them  or  that  they  hear  others  speak,  and  echopraxia, 
the  repetition  of  movements  which  are  made  in  their  presence.  On 
the  other  hand  there  may  be  a  marked  degree  of  muscular  tension, 
the  patient  maintaining  fixed  attitudes  with  the  muscles  thrown  into 
a  condition  of  rigid  contraction.  The  limbs  and  body  are  stiff  and 
immobile  and  resist  any  effort  at  bending.  Patients  in  this  condition 
are  positively  negativistic,  withdrawing  from  all  approaches,  refusing 
any  cooperation  with  the  nurse  in  attempting  to  dress  or  undress  or 
feed  them,  and  show  a  tendency  to  react  by  doing  the  opposite  of 
what  is  expected  of  them.  In  this  condition  of  stupor  the  patients 
may  appear  to  be  quite  disoriented  and  have  no  knowledge  of  what 
is  going  on  about  them.  If  they  are  watched,  however,  they  may  be 
seen  at  times  to  show  evidences  of  paying  attention  to  their  environ- 
ment, and  not  infrequently  when  the  stupor  passes  they  are  able  to 
give  a  fairly  good  account  of  the  things  that  happened  during  it,  but 
show  no  capacity  for  explaining  their  strange  conduct. 

In  the  opposite  condition  of  catatonic  excitement  there  are  marked 
degrees  of  activity,  constant  talkativeness  and  noisiness,  sometimes 
destructive  and  impulsive  tendencies  manifested  by  breaking  windows 
or  attacking  those  about  them,  but  in  general  showing,  as  does  the 
speech,  a  marked  lack  of  coherence.  Both  the  productivity  and  the 
activities  of  the  patient  fail  to  show  any  clear  goal,  although  some 
patients  show  what  very  closely  approaches  to  the  flight  of  ideas  of 
the  manic.  Catatonic  excitement  may  reach  a  very  high  grade,  mani- 
festing itself  by  wildly  deliroid  reactions,  constant  motor  unrest  and 
sleeplessness,  a  rapid  failure  in  nutrition,  a  veritable  status  catatoni- 
cus.  Some  of  these  catatonic  cases  show  hysteriform  or  epileptiform 
seizures,  and  death  occasionally  results  in  these  higher  grades  of 
excitement. 

The  chronic  conditions  tend  to  show  well-defined  mannerisms,  such 
as  the  peculiar  attitudes  of  the  body,  especially  clumsy  ways  of  holding 
the  spoon  or  the  fork  in  eating,  meaningless  grimaces,  odd  ways  of 
walking,  such  as  sliding  the  foot  back  and  forth  two  or  three  times 
before  starting  off,  and  other  ceremonials  for  initiating  movements. 
They  are  characteristically  stiff,  awkward,  clumsy,  and  inaccessible. 


CATATONIA 


697 


and  usually  indifferent  to  their  surroundings,  and  apparently  emotion- 
ally dull,  though  given  at  times  to  emotional  outbreaks  without 
apparent  cause.  All  of  these  psychical  symptoms  of  course  must 
be  considered  as  having  some  psychological  meaning.  In  harmony 
with  what  has  been  said  before  both  the  types  of  increased  suggesti- 
bility and  catatonic  rigidity  are  ways  of  shutting  out  the  world  of 
reality,  whereas  the  peculiar  automatisms  can  sometimes  be  traced 
to  their  meanings,  as  in  the  case  before  mentioned  of  the  young  woman 
who  kept  constantly  pounding  her  hand  with  her  clenched  fist. 

Physical  SymjJtonis. — The  general  appearance  of  the  catatonic  forms 
of  precox  indicate  more  than  in  the  other  varieties  the  presence  of 
definite  physical  illness.     Exaggeration  of  the  tendon  reflexes,  lower- 


FiG.  309. — Dementia  precox;  mannerism. 


Fig.  310. — Dementia  precox; 
mannerism. 


ing  of  cutaneous  sensibility,  vasomotor  disturbances,  cold  and  cyanosed 
extremities,  widel}'  dilated  pupils,  disturbances  of  secretion,  and  loss 
of  weight  are  frequently  observed.  It  is  this  group  of  cases  that 
have  led  most  distinctly  to  a  toxic  theory  of  etiology  and  caused  the 
precox  cases  to  be  thought  of  in  connection  with  disturbances  of 
metabolism  due  to  changes  in  the  internal  secretions. 

IV.  Paranoid  Forms. — In  the  paranoid  cases  there  is  a  much  more 
efficient  effort  at  creating  a  coherent  and  logically  connected  series 
of  delusions  and  associated  hallucinations.  In  these  cases  there  is 
much  less  outward  evidence  of  the  dilapidation  and  emotional  indif- 
ference that  have  led  to  the  belief  in  the  presence  of  a  permanent 
mental  impairment,  a  dementia. 


698  DEMENTIA   PRECOX  GROUP 

These  patients,  originally,  many  of  them  at  least,  were  grouped 
with  the  paranoias,  but  under  the  influence  of  Kraepelin  that  group 
of  paranoid  conditions  which  showed  a  tendency  toward  progressive 
deterioration  were  included  in  the  dementia  precox  classification. 
All  degrees  of  intervening  possibilities,  however,  occur  so  that  one  may 
get  a  fairly  well-knit  delusional  system  in  a  patient  who  deteriorates 
quite  rapidly,  whereas  other  patients  maintain  their  intellectual 
integrity  over  a  period  of  years.  The  delire  chronique  of  Magnan  is 
generally  considered  to  be  dementia  paranoides,  and  this  group  of 
cases  in  general  have  been  recently  included  by  Kraepelin  under  the 
designation  of  paraphrenia.  Some  authors  today,  more  particularly 
Bleuler,  are  inclined  to  think  that  perhaps  the  so-called  true  paranoias 
are  only  attenuated  forms  of  dementia  precox. 

The  delusional  system  in  these  cases  must  be  conceived  as  a  com- 
promise formation  and  as  essentially  wish-fulfiling,  and  is  characteris- 
tically sexually  colored.  One  such  patient,  a  middle-aged  woman, 
was  persistently  persecuted  by  a  man  who  accused  her  of  leading  an 
immoral  life  to  such  an  extent  that  she  finally  went  to  a  physician  to 
be  examined  to  prove  her  virginity.  All  sorts  of  vulgar  remarks  were 
constantly  made  about  her,  she  was  referred  to  by  the  voices  as  the 
widow  of  this  man.  The  patient  was  a  devout  Catholic  and  the  man 
was  a  Protestant,  a  perfectly  understandable  reason  for  a  severe 
emotional  conflict,  which  in  her  delusional  system  unloads  itself  upon 
the  man  rather  than  acknowledging  its  true  origin.  This  whole  con- 
flict arose  at  the  time  of  the  death  of  her  brother  and  it  is  significant 
that  in  her  delusions  she  believed  that  she  had  received  letters  accusing 
her  of  incestuous  relations  with  her  brothers,  of  having  become  preg- 
nant by  them,  and  of  destroying  the  pregnancy.  These  letters  were 
addressed  to  her  as  the  wife  of  her  several  brothers.  Here  one  sees 
an  infantile  determiner  for  the  delusional  system  in  the  love  which 
she  entertained  for  her  brothers  and  which  later  was  transformed, 
under  the  influence  of  the  conflict,  into  delusions  of  an  incestuous 
nature.  Finally,  there  was  a  whole  crowd  of  people  who  were  conspir- 
ing against  her  and  whose  object  it  was  to  kill  her.  Such  delusional 
systems  can  without  much  difficulty  be  seen  to  be  expressions  in 
distorted  form  of  the  conflict.  She  has  never  been  able  to  get  away 
from  her  infantile  attachment  to  the  members  of  her  family  and 
establish  herself  upon  her  own  feet,  and  these  attachments  hold  her 
back,  produce  a  withdrawal  from  the  outer  world,  a  regression  into 
the  world  of  phantasy,  with  a  tendency  toward  what  may  be  termed 
psychic  death,  or  an  absolute  lack  of  efficient  reaction  to  reality,  and 
this  is  symbolized  by  the  gang  of  persecutors  who  are  bent  upon  her 
destruction. 

V.  Mixed  and  Atypical  States.— AW  of  these  forms  of  dementia 
precox  so  far  described  are  simply  variants,  in  accordance  with  the 
present  viewpoint,  of  one  disease  trend,  and  so  it  is  not  strange  that 
it  should  be  found  that  there  are  no  hard-and-fast  lines  separating 


NATURE  OF  DEMENTIA  PRECOX  699 

one  from  the  other.  In  consequence  it  is  not  infrequent  to  see  transi- 
tory or  mild  motility  disturbances  in  cases  that  are  essentially  hebe- 
phrenic, or  to  see  fairly  well-defined  paranoid  delusional  formations 
in  either  the  hebephrenic  or  the  catatonic  varieties. 

As  to  the  atypical  forms,,  there  has  been  mentioned  and  described 
under  dementia  simplex  the  larvated  and  abortive  types,  the  "formes 
frustes."  Tliese  are  probably  much  more  frequent  than  ordinarily 
supposed.  For  example,  Wilmanns,  in  a  study  of  127  vagabonds, 
found  66  cases  of  dementia  precox,  while  undoubtedly  it  is  not  infre- 
quent for  these  abortive  forms  to  be  considered  as  cases  of  constitu- 
tional defect.  This  difficulty  becomes  very  much  more  aggravated 
when  it  is  remembered  that  attacks  of  dementia  precox  may  occur 
in  childhood,  according  to  Vogt  as  early  as  five  years  of  age,  while  a 
little  later,  nearer  to  the  period  of  puberty,  they  are  not  so  uncommon. 
In  these  cases  of  course  mental  development  is  interfered  wdth  and 
remaining  upon  a  lower  level  gives  the  impression  of  imbecility.  The 
same  difficulty  arises  in  a  somewhat  aggravated  form  when  dementia 
precox  develops  in  young  persons  already  of  defective  make-up. 
Here,  of  course,  the  differentiation  is  no  longer  possible,  because  it 
must  be  realized  that  both  conditions  of  defect  and  precox  are  present 
in  the  same  individual. 

Pathology. — There  is  httle  that  is  distinctive  in  the  pathology  of 
precox.  It  is  probable  that  during  the  life  of  the  individual  disturb- 
ances that  are  shown  in  the  general  physical  manifestation  of  the 
disease  occur  at  the  biochemical  leveh  Beginning  degenerative 
changes  in  the  vessels  are  sometimes  found,  and  tuberculosis  is  quite 
frequent,  but  is  easily  understandable  as  being  dependent  upon  the 
general  lowered  physical  condition  and  inactivity  of  these  patients, 
coupled  with  their  bad  habits  and  the  necessity  of  their  rather  close 
confinement.  Such  changes  as  are  found  in  the  nerve  cells  are  of  a 
degenerative  character,  with  evidences  of  neuronophagia  and  with 
perhaps  ameboid  glia  cells.  The  acute  cases  of  catatonic  excitement 
w^hich  end  fatally,  the  so-called  catatonic  "Hirntod,"  show  a  certain 
amount  of  evidence  of  degeneration,  more  particularly,  however, 
evidences  of  a  chemical  nature  in  the  form  of  certain  lipoid  elements 
in  the  cortex,  which  seem  to  be  products  of  disintegration. 

Southard  has  found  certain  anomalies  in  precox  brains  w^hich 
appear  to  be  of  the  nature  of  aplasias  or  ageneses.  These  agenetic 
or  aplasic  areas  appear  to  be  grouped  more  or  less  in  correspondence 
with  the  three  main  types  of  the  disease.  The  frontal  region  is  charac- 
teristically involved,  while  he  has  described  a  cerebellar  group  corre- 
sponding to  the  catatonic  variety,  while  the  profound  emotional 
disturbances  he  thinks  are  due  to  lesions  in  the  deep  layers  of  the 
cortex  which  have  no  direct  motor,  sensory,  or  perhaps  associational 
relations. 

Nature  of  Dementia  Precox. — From  the  description  of  dementia 
precox  up  to  this  point  it  will  be  seen  that  it  presents  correlations  on 


700 


DEMENTIA   PRECOX  GROUP 


the  one  hand  to  the  more  distinctly  so-called  functional  types  of 
disorders,  such  as  the  psychoneuroses,  and  on  the  other  hand  to  the 
more  distinctly  organic  diseases.  It  has  been  seen  that  it  was  possible 
to  formulate  all  of  the  mental  symptoms  in  the  same  way  that  they 
are  formulated  in  the  psychoneuroses.     In  other  words,  the  mental 

symptoms  are  capable  of  interpretation 
solely  at  the  psychological  level.  On  the 
other  hand,  for  an  understanding  of  the 
whole  disease  process  it  must  not  be  lost 
sight  of  that  recent  investigations  are  tend- 
ing to  show  more  and  more  that  there  are 
distinct  biochemical  disturbances  during 
life  and  pathological  changes  are  being 
found  after  death.  In  any  case  the  acute 
cases  that  lead  to  death  must  be  con- 
ceived as  having  profound  bodily  changes 
correlated  with  the  psychic  symptoms. 

The  formulation  of  the  disease  from 
either  standpoint  alone  must  of  necessity 
be  unsatisfactory.  For  example,  the  theory 
which  makes  the  etiology  toxic  fails  utterly 
to  give  a  comprehensive  idea  of  why  the 
mental  manifestations  take  the  particular 
form  they  do,  why  for  example,  in  the 
case  already  cited,  the  hallucinatory  voice 
should  be  that  of  the  young  woman  that 
the  patient  had  seduced,  telling  him  to 
lead  a  decent  life.  This  is  surely  the  pa- 
tient's self-critique  appearing  in  hallucina- 
tory form. 

It  would  seem,  especially  in  view  of 
the  type  of  archaic  reaction  which  has 
been  described  and  which  is  found  quite 
frequently  in  precox,  that  the  schizophrenic 
splitting  of  the  personality  is  more  severe, 
more  profound,  than  in  the  so-called  func- 
tional disorders,  in  other  words,  that  it 
goes  to  deeper  levels,  that  the  infantile 
and  the  archaic  in  the  individual  have 
been  less  efficiently  sublimated  and  so 
gotten  away  from,  and  therefore  they  con- 
trol more  effectively  the  thoughts  and  acts. 
At  any  rate,  it  seems  quite  evident  that  the  great  difficulty  of  concep- 
tion here  is  due  in  part  to  the  quite  arbitrary  separation  of  the  indi- 
vidual into  two  distinct  and  mutually  exclusive  parts,  namely,  mind 
and  body.  The  dualistic  hypothesis  that  conceives  of  the  mental  and 
the  physical  going  side  by  side  without  mutual  interference  or  interac- 


FiG.  311. — Phantastic  paranoia. 


NATURE  OF  DEMENTIA  PRECOX  701 

tion  is  responsible  for  such  a  conception.  From  the  medical  standpoint 
such  a  concept  seems  entirely  unnecessary,  for  one  is  met  at  every 
turn  with  the  intimate  relations  constantly  maintained  between  the 
two,  and  therefore  come  naturally  to  consider  the  human  being  as  a 
biological  luiit  presenting  types  of  reactions  that  at  one  extreme  are 
predominantly  psychic,  while  at  the  other  they  are  predominantly 
physical,  but  which  present  every  grade  of  intermediary  type.  The 
severe  mental  disturbance  of  the  nature  of  anxiety  that  goes  with 
certain  cardiac  and  pulmonary  diseases  is  well  known,  as  are  also  the 
physical  upsets,  particularly  the  gastro-intestinal,  that  are  associated 
with  certain  mental  conditions  such  as  worry.  The  interrelations 
between  the  mental  and  the  physical  are  a  matter  of  daily  observation. 
Occasionally,  however,  they  are  very  pronouncedly  emphasized.  A 
chronic  patient  who  had  been  for  many  years  in  a  hospital,  working 
daily  at  out-door  labor  was  suddenly  seized  with  a  violent  impulse  in 
which  he  attacked  all  about  him.  He  seized  a  heavy  iron  bar,  killed 
two  people  and  injured  another,  and  ran  head-long  and  wildly  without 
direction  into  the  woods.  He  was  finally  cornered,  and  in  the  process 
of  securing  him  he  was  shot  by  a  farmer  with  a  load  of  buckshot,  none 
of  which,  however,  penetrated  further  than  through  the  skin  and 
produced  no  serious  wounds.  He  was  brought  back  to  the  hospital, 
incoherent,  mumbling  and  trembling,  showing  all  the  evidences  of  a 
tremendous  emotional  upset.  The  shot  were  picked  out  of  his  skin, 
the  wounds  were  dressed,  and  he  was  put  in  bed.  Up  to  that  time  he 
had  been  a  strong,  physically  healthy  negro.  He  never  left  his  bed 
again,  and  approximately  a  year  afterward  he  died,  having  developed 
an  acute  tuberculosis.  Such  cases  as  these  demonstrate  the  necessity 
of  considering  the  human  being  as  a  unit  and  not  endeavoring  to  draw 
hard-and-fast  lines  of  distinction  between  the  mind  on  the  one  hand 
and  the  body  on  the  other. 

In  our  present  state  of  knowledge,  however,  we  are  quite  unable 
to  make  any  specific  correlation  between  the  physical  findings  and 
the  mental  symptoms,  while  on  the  other  hand  it  is  quite  possible 
to  express  the  symptomatology  of  the  disease,  to  describe  it,  to,  so  to 
speak,  reconstruct  the  psychosis  purely  in  psychological  terms.  For 
the  present,  therefore,  the  disease  must  be  described  psychologically, 
and  the  explanation  of  the  mental  symptoms  must  be  sought  psycho- 
genetically,  without,  however,  forgetting  that  there  are  certain  somatic 
changes  which  are  pretty  generally  attached  to  the  symptomatology 
of  the  disease  process  and  which  must  ultimately  be  made  to  fit  into 
the  general  rubric  before  a  complete  understanding  of  the  entire 
situation  is  had. 

On  the  psychological  side,  then,  dementia  precox  is  seen  to  be  a 
certain  type  of  reaction  to  a  mental  conflict,  resulting  in  a  splitting 
of  the  psyche  and  the  outcrop  of  unconscious  mental  trends  to  the 
surface  of  the  mental  life.  The  patient  is  confronted  with  a  situation 
to  which  he  cannot  adequately  adjust,  which  is  absolutely  inaccept- 


702 


DEMENTIA   PRECOX  GROUP 


able  and  impossible,  and  he  is  therefore  driven  away  by  his  incapacity 
to  assimilate  it  and  cast  back  upon  himself.  The  battle  of  the  opposing 
forces  produces  the  disease  picture  which  is  the  outward  evidence  of 
^the  effort  on  the  part  of  the  individual  to  reach  a  solution  of  the 
difficulty.  The  symptoms  are  the  result  of  the  appearance  of  the  uncon- 
scious trends  distorted  and  disguised  as  they  are  in  dream  formations. 
Jung  has  especially  noted  this  similarity  to  the  dream  state  and  would 
consider  precox  as  a  sort  of  waking  dream  or  dream  from  which  the 
patient  does  not  awake,  the  dream  picture  being  fixed,  as  it  were, 
by  another  element  in  the  situation,  the  toxin,  which  acts  like  the  fixing 
agent  in  the  photographic  process.  It  is  seen  that  the  conception 
of  the  disease  as  being  from  the  beginning  and  fundamentally  a 
dementia  must  be  modified,  if  by  dementia  is  meant  a  permanent 

mental  impairment,  a  mental  loss, 
which  is  thought  of  in  the  same 
terms  as  a  loss  of  tissue  from  a 
wound.  It  would  appear  from  the 
description  of  the  mechanisms  that 
have  been  given  that  at  least  in  the 
early  stages  of  the  disease  there  is 
nothing  at  all  corresponding  to  such 
a  permanent  loss;  that  what  has 
happened  has  been  a  disintegration, 
a  falling  apart  of  the  component 
parts  of  the  psyche  and  a  shifting 
of  relative  positions,  more  particu- 
larly a  shifting,  or  to  use  the  tech- 
nical term,  a  displacement  of  the 
emotional  content  of  certain  idea 
constellations.  The  dementia,  there- 
fore, which  has  been  described  as 
such,  is  at  least  to  this  extent  a 
misnomer.  From  the  point  of  view, 
however,  that  this  disintegration  and  resulting  impairment  is  either 
permanent  or  tends  to  be,  it  has  a  meaning. 

The  psychological  side  of  the  situation,  however,  we  have  seen  is 
not  all.  There  are  certain  physical  changes  in  the  course  of  the  disease, 
and  certain  pathological  findings.  So  far  as  our  observation  goes, 
however,  the  etiological  factors  lie  almost,  if  not  quite  entirely,  in  the 
mental  sphere,  and  one  must  therefore  conceive  of  the  physical  changes 
as  superadded.  This  is  a  possibility  which  was  well  illustrated  by  the 
case  already  quoted  of  the  man  who  died  after  a  tremendous  emotional 
explosion  during  which  he  killed  two  people.  When  the  psychic 
splitting  is  profound  and  when  it  is  of  considerable  duration  it  is  quite 
understandable  that  it  should  unloose  bits  of  physiological  mechanism 
and  thus  produce  the  physical  changes  found.  From  the  descriptions 
of  the  mechanisms  in  the  psychoneuroses,  taken  in  connection  with 


Fig.  312.— The  fetal  attitude  assumed 
in  severe  grades  of  regression.  This  at- 
titude was  maintained  most  of  the  time 
for  years,  the  patient  seeking  the  dark- 
est corner  of  the  ward. 


WAYS  OF  GETTING   WELL  703 

the  discussion  of  the  vegetative  nervous  system,  it  may  be  seen  that 
constanth^  operative  psychic  disturbances  are  capable  of  producing 
the  physical  changes.  Compare  Crile's  study  of  the  emotions  and 
Cannon's  work  on  the  relation  of  the  psyche  to  the  gastro-intestinal 
functions  where  it  may  be  seen  that  both  surgeon  and  physiologist 
are  forced  to  put  the  psychic  factor  in  the  foreground. 

Ways  of  Getting  Well. — With  the  concept  of  the  disease  process  which 
has  been  previously  elaborated,  what  is  the  significance  of  the  three 
main  types  of  precox  which  are  found  clinically?  Bertschinger^  has 
recently  made  an  admirable  study  of  the  process  of  recovery  in  precox 
and  his  discussion  of  the  nature  of  the  conflict  and  the  way  of  adjusting 
is  illuminating. 

The  three  clinical  types  of  the  disease  are  the  expression  of  the 
interplay  of  the  two  factors,  the  conflict  and  the  reaction,  the  severity 
of  the  former  and  the  efficiency  of  the  latter  determining  the  outcome 
in  the  individual  case.  The  degree  of  confusion  in  the  acute  onset 
would  then  be  an  expression  of  the  completeness  with  which  the 
patient  was  driven  back  from  reality  and  the  dominance  of  the  uncon- 
scious trends.  Conditions  of  moderate  confusion  with  capacity  for 
adequate  reaction  to  reality  at  times,  or  under  the  special  stimuli  of, 
for  example,  questions,  show  that  the  patient  still  has  a  certain  grip 
upon  the  real  world  and  is  making  an  effort  at  least  to  retain  it.  Certain 
other  cases  of  quite  clear  consciousness  with  complete  orientation  show 
a  very  adequate  grasp  upon  reality,  and  these  patients,  to  the  casual 
observer,  often  seem  quite  natural.  In  such  patients,  however,  one 
will  notice  interference  of  thought,  hesitations  in  the  course  of  conver- 
sations, stutterings  and  stammerings  over  certain  points,  the  evidences 
in  other  words,  of  complex  interferences,  and  the  patients  will  complain 
that  from  time  to  time  their  minds  seem  to  be  absolutely  blank. 
These  thought  deprivations  and  saltatory  associations  are  the  expressions 
of  reactions  to  buried  complexes,  so  that  in  these  patients  there  is  a 
fairly  adequate  grasp  upon  reality  for  ordinary  purposes  at  least,  with 
only  spasmodic  influences  from  relatively  restricted  areas. 

In  general,  then,  the  hebephrenic  type  may  be  seen  to  be  a  reaction 
to  the  conflict  which  is  essentially  inadequate  and  inefficient.  In  the 
acute  stages  the  patient  may  be  absolutely  overwhelmed  by  the 
conflict,  disoriented,  and  confused.  Later  on  the  progressive  disinte- 
gration and  dilapidation  of  thought  indicates  the  slowly  progressive 
conquering  of  the  capacity  for  adjustment  to  reality  by  the  invasion 
into  consciousness  of  the  unconscious  trends. 

The  catatonic  type  represents  a  somewhat  different  form  of  reaction. 
Here  the  patient  is  oftentimes  suddenly  overwhelmed  by  the  conflict, 
as  under  circumstances  of  accident  or  sudden  and  severe  shock.  No 
attempt  at  adjustment  is  made  at  first,  but  the  whole  situation  is 
actively  and  definitely  shut  out.     Here  there  is  an  active  effort  on 

1  Heilungsvorgange  bei  Schizophrenen,  Allg.  Zeits.  Psychiat.,  Band.  Ixviii,  Heft  2. 
Translated  in  Psychoanalytic  Review,  1915. 


704  DEMENTIA   PRECOX  GROUP 

the  part  of  the  individual  to  exclude  the  offending  tendencies,  and 
when  this  succeeds  recovery  takes  place  as  the  result,  so  to  speak,  of 
the  encapsulation  of  the  objectionable  material,  and  its  exclusion  from 
consciousness.  This  form  of  the  disorder  is  the  most  acute,  and  the 
recovery  is  equally  most  apt  to  be  prompt,  and  it  will  be  seen  from  this 
explanation  why  this  is  so. 

In  the  paranoid  form  of  the  disorder  the  reaction  is  much  more 
efficient  than  in  the  hebephrenic  variety,  and  in  some  respects  less 
efficient  than  in  the  catatonic.  Here  the  individual  takes  a  flight 
into  a  psychosis,  and  the  delusions  are  the  expression  of  a  compromise 
between  the  opposing  psychic  trends.  Unable  to  live  in  the  real  world 
the  patient  succeeds  in  inventing  a  world  in  which  he  can  live  and 
having  invented  it  he  succeeds  in  getting  along  fairly  well  without 
noticeable  deterioration.  The  conflict  in  these  cases  tends  to  become 
stationary  after  the  development  of  the  delusional  world. 

Bertschinger  has  more  especially  defined  the  ways  of  getting  well 
by  pointing  out  that  the  patient  in  recovering  may,  as  the  result  of 
his  conflict  to  which  hie  cannot  adjust,  find  a  compromise  by  changing 
himself  and  interpreting  the  world  of  reality  in  terms  of  his  morbid 
phantasy,  or  by  translating  the  world  of  his  phantasy  into  terms  of 
external  experience.  And  so  one  would  find  on  the  one  hand  delusions 
of  grandeur  which  are  a  compromise  formation  and  serve  to  change 
the  individual  so  that  he  may  be  better  satisfied  with  life,  and  on 
the  other  hand  one  finds  delusions  of  persecution,  the  delusions  of 
infiuence  from  the  outer  world,  that  serve  to  change  the  outer  world 
in  conformity  with  the  patient's  complexes.  These  outside  influences 
are  but  the  reflections  back  upon  the  patient  of  his  failures  to  get  from 
the  world  what  he  wants,  and  they  are  consequently  felt  as  malign 
and  destructive  influences.  Another  method  of  getting  well  is  that 
already  described  of  the  catatonic,  the  shutting  out  and  encapsulation 
of  the  conflict  in  a  circumscribed  amnesia. 

In  many  cases  conversions  into  bodily  symptoms,  such  as  are  found 
in  hysteria,  are  found.  Another  method  of  getting  well  is  by  living 
through  a  series  of  imaginary  experiences  which  brings  the  complex 
to  a  logical  conclusion.  For  example:  A  young  Japanese  woman 
was  overwhelmed  by  the  sad  news  that  five  members  of  her  family 
had  been  killed  in  battle.  She  passed  instantly  into  a  dreamy  state  of 
consciousness,  went  on  with  the  work  of  the  household  just  as  if  all 
five  were  members  of  it,  made  their  beds,  set  their  places  at  table  and 
acted  in  every  way  as  if  they  were  alive  and  present.  Finally  she, 
so  to  speak,  let  one  of  them  die  and  then  another  and  another  until 
finally  she  had  compassed  the  death  of  all  five,  after  which  she  awoke 
from  her  dream-like  state  and  was  well.  She  had  succeeded  in  an 
efficient  reaction  to  the  situation  by  its  attenuation,  extending  it  over 
a  considerable  period  of  time.  Finally,  a  certain  number  of  patients 
get  well  by  the  final  domination  of  the  reality  motive,  with  a  resulting 
correction  of  their  delusional  phantasies. 


COURSE  AND  PROGRESS  705 

Course  and  Progress. — As  will  be  seen  from  the  description  of  the 
disease  the  catatonic  form  is  more  apt  to  be  acute  in  onset  and  it  has 
the  best  prognosis,  while  the  hebephrenic  form  and  the  simple  dement- 
ing varieties  tend  to  progressive  deterioration,  and  the  paranoid  form 
tends  to  remain  stationary  without  material  deterioration.  A  few 
of  the  hebephrenics  get  well,  more  of  the  catatonics  recover,  but  all 
of  these  cases  are  liable  to  recurrent  attacks.  According  to  a  recent 
study  of  Zablocka^  of  515  cases,  60  per  cent,  proceeded  to  light,  18  per 
cent,  to  medium,  and  22  per  cent,  to  severe  deterioration. 

In  the  catatonic  cases  that  recover  there  is  usually  an  appreciable 
change  in  the  individual.  The  encapsulation  of  the  conflict  means 
that  the  recovery  has  taken  place  by  a  sacrifice  of  a  certain  portion  of 
the  personality.  The  portion  of  the  personality  in  which  the  conflict 
is  resident,  so  to  speak,  has  had  to  be  cut  out  or  walled  off,  and  the 
energies  from  this  region  are  no  longer  available  by  the  individual. 
This  loss  is  quite  characteristically  shown  in  certain  changes  of  char- 
acter, indicating  that  the  individual  has  gotten  well,  but  has  paid  a 
certain  price  for  that  result. 

The  cases  that  do  not  get  well  tend  to  regress  to  lower  levels,  and 
quite  characteristically,  in  institutions  at  least,  they  finally  reach  a 
level  on  which  they  can  maintain  themselves  without  sinking  further. 
In  this  condition  they  remain  stationary  for  long  periods  of  time. 
Quite  often  patients  brought  to  an  institution  in  a  very  much  disturbed 
condition  settle  down  quite  promptly  under  the  simpler  conditions 
of  institution  life,  while  in  the  outer  world  the  precox  cases  tend  to 
gravitate  into  the  ranks  of  the  hobo,  the  prostitute,  and  the  petty 
criminal.  Here  they  finally  find  their  level  and  get  along  after  a  fashion. 

Acute  and  severe  grades  of  regression,  in  which  the  splitting  goes  to 
the  very  foundation  of  the  personality,  often  expresses  themselves 
by  suicidal  attempts  and  sometimes  by  homicidal  attempts.  The 
possibility  of  such  attempts  may  sometimes  be  foreseen  in  the  dreams. 

Remissions  are  quite  the  rule  and  come  about  under  circumstances 
which  reanimate  the  conflict.  Often  patients  get  along  very  well  in 
an  institution,  but  become  upset  shortly  after  going  back  to  the 
conditions  under  which  the  conflict  developed. 

The  course  of  the  disease  is  irregular  and  prediction  is  quite  impos- 
sible. In  a  general  way,  however,  one  gets  clues  from  keeping  in 
mind  the  fact  that  the  conflict  is  between  the  reality  motive  and  the 
phantasy  formation,  and  by  watching  the  interplay  of  these  two 
motives  one  gets  an  idea  as  to  whether  the  reality  motive  is  gaining 
or  losing  ground. 

The  question  of  a  recovery  is  a  mooted  one,  but  in  general  it  is 
conceded  that  the  patients  that  recover  do  present  to  careful  analysis 
certain  residuals.  Of  course,  however,  these  residuals  may  be  of  any 
degree  and  may  be  so  slight  as  to  not  necessarily  impair  the  individuals 

1  Zur  Prognosestellung  bei  der  Demeatia  Prgecox,  Allg.  Zeit.  f.  Psychiat.,  Band.  Ixv. 
45 


706  DEMENTIA   PRECOX  GROUP 

in  the  position  in  life  which  they  may  occupy.  It  is  possible  that  true 
recovery  may  take  place  with  a  resolution  of  all  the  symptoms,  but  if 
this  is  so  it  is  probably  the  exception. 

Diagnosis. — ^The  diagnosis  of  dementia  precox,  while  comparatively 
easy  in  the  well-defined  and  the  advanced  cases,  becomes  a  matter  of 
great  difiiculty  during  the  early  history  of  the  illness  or  in  cases  in  which 
the  symptoms  are  mild. 

The  manic-depressive  psychosis  presents  one  of  the  characteristic 
difficulties.  In  this  psychosis  there  is  usually  a  history  of  repeated 
attacks  without  deterioration.  It  must  not  be  forgotten,  however, 
that  precox  itself  frequently  presents  a  similar  history  and  that  if 
deterioration  is  present  it  may  not  be  prominently  in  evidence.  The 
depression  which  so  frequently  ushers  in  a  precox  attack  may  easily 
be  mistaken  for  a  depression  of  the  manic-depressive  psychosis.  The 
manic-depressive  retardation  is  similar  in  its  outward  appearances 
to  the  indifference  and  perhaps  lighter  degrees  of  negativism,  particu- 
larly the  inaccessibility  of  the  precox.  The  manic-depressive  is  more 
apt  to  have  delusions  of  a  self-accusatory  type  than  the  precox,  and 
the  latter' s  delusions  are  more  apt  to  be  grotesque.  They  more 
frequently  are  evident  distortions  and  symbolisms.  The  pressure  of 
activity  of  the  manic-depressive  resembles  the  activity  of  the  cata- 
tonic. In  the  former,  however,  the  activity,  although  rapidly  changing 
as  to  its  object,  is  characteristically  addressed  to  some  purpose,  while 
in  the  catatonic  the  activity  is  more  diffuse  and  has  less  direction.  It 
is  more  incoherent. 

In  the  early  stages  the  mild  depression  of  the  precox  may  simulate 
that  of  a  neurasthenic,  or  the  agitated  depression  may  simulate  that 
of  an  anxiety  neurosis.  In  both  instances  the  precox  is  more  apt  to 
show  grotesque  delusions  and  conduct  disorders  of  a  bizarre  nature, 
such  as  tearing  his  clothes,  self-mutilation,  or,  on  the  other  hand, 
characteristic  negativistic  symptoms,  such  as  retaining  the  saliva  or 
the  urine,  withdrawing  from  efforts  addressed  to  assist  him,  refusing 
to  cooperate  in  changing  his  clothes,  the  refusal  of  food  and  the  like. 
It  must  not  be  forgotten,  however,  that  the  etiological  factors  of  the 
actual  neuroses  may  operate  in  the  same  person  who  breaks  down  as  a 
precox,  and  that  therefore  neurasthenic  and  anxiety  symptoms  may 
be  present  as  expressions  of  these  etiological  factors.  It  is  important 
to  keep  such  a  possibility  as  this  in  mind  when  it  comes  to  the  matter 
of  treatment. 

It  is  still  a  mooted  question  whether  there  are  not  conditions  inter- 
mediate between  precox  and  manic-depressive  psychosis  which  partake 
somewhat  of  the  characteristics  of  both.  We  know  there  are  a  con- 
siderable number  of  cases  in  which  the  difficulties  of  diagnosis  are  very 
great.  Flight  of  ideas  may  be  quite  typical  in  precox,  for  example, 
while  the  depression  of  the  precox  may  resemble  very  closely  that 
of  the  manic-depressive.  The  difficulties  are  greatest  with  that  class 
of  manic-depressive  psychoses  known  as  the  mixed  states. 


DIAGNOSIS  707 

Epileptiform  and  hysteriform  episodes-  may  lead  to  a  diagnosis 
of  epilepsy  or  hysteria.  It  must  not  be  forgotten,  however,  that  it  is 
possible  to  have  precox  complicated  with  epilepsy  and  that  in  dementia 
precox  all  of  the  symptoms  and  characteristic  mechanisms  that 
are  found  in  hysteria  may  be  found. 

In  the  infection  and  exhaustion  psychoses  the  differentiation  may 
be  very  difficult  and  it  is  necessary  in  such  patients  often  to  wait  for  a 
considerable  time  until  the  subsidence  of  the  infection  and  then  see 
whether  the  case  clears  up,  as  it  usually  does  if  it  is  a  simple  infection 
psychosis.  One  has  to  be  very  careful  in  making  a  prognosis  in  cases 
of  this  character.  Not  infrequently  cases  of  so-called  puerperal 
insanity  are  really  cases  of  precox  which  have  been  precipitated  by  the 
circumstances  of  the  puerperal  period,  loss  of  blood,  prolonged  labor, 
infection,  or  the  mental  stress  incident  to  an  illegitimate  pregnancy. 

From  paresis  the  differentiation  can  now  be  made  by  the  laboratory 
methods  at  our  disposal.  One  must  not  forget,  however,  that  the 
precox  may  have  syphilis  and  therefore  a  positive  Wassermann  in  the 
blood  serum.  In  fact,  not  a  few  do  show  this  reaction,  and  it  is  quite 
readily  conceivable  that  the  presence  of  an  uncured  syphilis  may 
well  be  a  precipitating  factor  in  the  outbreak  of  the  psychosis  in  a 
certain  proportion  of  cases. 

From  the  toxic  psychoses,  particularly  from  alcoholic  deterioration, 
the  differentiation  is  often  quite  difficult.  It  must  be  borne  in  mind 
in  this  connection  that  the  relatively  normal  man  deteriorates  very 
slowly  from  the  use  of  alcohol,  while  one  finds  in  the  records  of  precox 
cases  who  have  indulged  in  alcohol  that  the  deterioration  has  come 
very  much  earlier.  In  addition  to  this  it  will  be  found  that  the  amount 
of  deterioration  in  the  precox  case  is  very  much  greater  than  could 
reasonably  be  explained  by  the  alcoholic  indulgence  of  the  patient. 
When  this  discrepancy  in  the  history  is  found  one  is  justified  in  suspect- 
ing that  one  is  dealing  with  a  fundamentally  more  serious  condition 
than  mere  alcoholism.  These  two  types  of  cases  show  a  character- 
istic type  of  defence  which  serves  to  separate  them,  the  one  from  the 
other.  In  both  instances  the  patients  tend  to  minimize  the  true 
etiological  factor  and  to  exaggerate  the  unimportant  one.  For  example, 
the  precox  case  will  always  exaggerate  the  amount  of  alcohol  he  has 
been  taking,  while  the  alcoholic  w^ill  always  minimize  it.  This  is  of 
course  only  roughly  true  but  it  may  be  helpful  in  sizing  up  patients. 

The  whole  question  of  alcoholic  deterioration  is  by  no  means  settled. 
Bleuler^  is  inclined  to  include  the  alcoholic  hallucinoses  in  the  dementia 
precox  group  and  at  least  it  must  be  acknowledged  that  many  alcoholic 
patients  after  repeated  attacks  of  acute  alcoholic  psychoses  undergo  a 
deterioration  which  is  precox  in  character. 

^  Dementia  Prsecox  oder  Gruppe  der  Schizophrenien  Franz  Deuticke,  Leipzig  u.  Wien, 
1911.  This  work  is  by  far  the  most  comprehensive  and  complete  treatment  of  the  whole 
dementia  precox  problem  extant.  An  excellent  review  of  the  work  by  August  Hoch 
will  be  found  in  the  Review  of  Neurology  and  Psychiatry,  June,  1912. 


708  DEMENTIA   PRECOX  GROUP 

The  difficulty  of  differentiating  between  defect  due  to  precox  and 
congenital  defect  has  already  been  mentioned  in  discussing  precox 
in  children,  and  precox  on  a  defective  basis.  A  material  help  in  making 
this  differentiation  is  an  inquiry  into  the  school  knowledge.  The  school 
knowledge  will  be  reasonably  well  retained  in  the  precox,  while  it  will 
not  have  been  acquired  in  the  defective. 

It  must  be  insisted  upon  that  in  the  beginning,  the  hebephrenic 
and  simplex  types  frequently  complain  of  numerous  minor  ailments. 
These  are  frequently  treated  by  the  general  practitioner  or  the  specialist 
on  the  basis  of  their  physical  rather  than  their  psychical  character. 
Such  patients  repeatedly  go  through  complicated  systems  of  treatment 
until  the  true  character  of  their  illness  has  become  evident,  when 
often  much  valuable  time  has  been  lost. 

Treatment. — Dementia  precox  has  generally  been  considered  to  be  a 
hopeless  condition  for  which  little  or  nothing  could  be  done.  This  is 
at  least  not  an  attitude  with  which  to  approach  a  patient,  and  when  we 
bear  in  mind  the  considerable  number  of  recoveries  that  take  place 
in  the  disease  it  is  hardly  an  attitude  that  is  warranted.  The  treat- 
ment, however,  must  of  necessity  be  very  difficult,  because  conditions 
that  have  to  be  met  are  multitudinous  and  range  all  the  way  from 
disturbances  at  the  lower  physiological  levels  through  distinctly 
psychological  problems  to  the  relation  of  the  individual  to  his  social 
milieu.    These  matters  may  be  taken  up  in  their  order. 

Treatment  of  Physical  Conditions. — Here  the  treatment  must  be 
practically  entirely  symptomatic.  The  bladder  and  rectum  must  be 
carefully  watched  where  there  is  negativistic  retention.  The  mouth 
must  be  kept  clean,  swabbed  out  with  listerine  or  some  other  antiseptic 
mouth  wash,  if  there  is  retention  of  saliva  or  food  particles,  otherwise 
putrefactive  changes  may  occur,  infections  of  the  gum  with  ulcerations 
may  result,  with  possible  complications,  such  as  pneumonia  and  death. 
Other  such  conditions  as  these  have  to  be  met  in  the  practical  and 
common  sense  way  and  need  little  special  comment;  for  instance, 
surgical  injuries,  such  as  self-mutilation,  bruises,  and  the  like  have  to 
be  met  in  the  usual  way,  while  it  is  especially  important  to  get  these 
patients  out  of  doors  and  not  permit  their  seclusive  tendencies  to 
further  the  development  of  tubercular  disease. 

At  the  present  time  a  good  deal  of  attention  is  being  paid  to  the 
internal  secretions.  These  may  be  investigated  in  the  individual  case, 
but  as  yet  their  beneficial  action  is  unproved. 

Treatment  of  the  Mental  Condition. — The  treatment  of  the  mental 
condition  resolves  itself  into  the  treatment  of  disturbances  at  lower 
and  higher  levels.  In  the  disturbances  of  the  lower  levels  we  have 
characteristically  the  excitements  and  the  stupors.  In  general  the 
treatment  of  the  excitements  should  be  by  hydrotherapy,  the  con- 
tinuous bath  or  the  pack,  according  to  the  individual  experience  of  the 
physician  or  the  convenience  of  the  hospital.  Restraint  either  physical 
or  chemical  should  be  avoided  if  possible,  resorting  to  drugs  only  in 


TREATMENT  709 

so  far  as  may  be  necessary  to  produce  a  sufRcient  amount  of  sleep. 
It  must  be  borne  in  mind  that  all  of  the  drugs  that  are  used  for  chemical 
restraint,  more  especially  those  belonging  to  the  belladonna  group,  are 
delirium-producing  and  therefore  tend  to  interfere  with  the  adjustment 
of  the  patient  to  reality.  Small  doses  of  atropin  are  not  contra- 
indicated  to  obtain  vegetative  nervous  system  control  of  certain 
annoying  somatic  symptoms. 

In  conditions  of  stupor  the  general  health  has  to  be  carefully  looked 
after.  The  patient  must  often  be  tube  fed,  bowels  and  bladder  require 
special  attention,  and  the  position  of  the  body  should  be  changed 
sufficiently  so  as  to  prevent  pressure  upon  any  portion  of  the  skin 
surfaces  or  a  tendency  to  hypostatic  congestion  of  the  lungs  in  weak- 
ened patients.  Cleanliness  and  regular  and  sufficient  feeding  are  the 
essential  things,  and  it  is  very  desirable  in  addition  to  wheel  the 
patient's  bed  out  upon  the  open  porch  where  he  can  have  abundance 
of  fresh  air,  if  this  is  possible. 

Treatment  at  the  higher  psychological  levels  has  the  same  things 
to  be  said  for  it  as  psychotherapy  has  for  the  psychoneuroses.  While 
it  cannot  be  expected  that  patients  can  be  cured  by  psychoanalytic 
treatment  as  they  are  when  suffering  from  the  psychoneuroses,  still 
it  must  be  remembered  that  all  of  the  symptoms  of  a  precox  case  are 
not  necessarily  at  the  same  level,  and  while  the  patient  may  not  be 
susceptible  of  a  cure,  still  many  of  the  symptoms  may  be  largely 
relieved,  if  not  dispersed  altogether.  Psychoanalysis,  therefore,  should 
be  used  and  even  where  the  therapeutic  effect  may  not  be  especially 
great,  still  it  furnishes  that  sort  of  information  about  the  patient  which 
it  is  essential  to  have  in  order  to  deal  with  him  intelligently.  One  must 
know  something  about  the  factors  that  produced  the  break-down,  one 
must  know  something  of  the  nature  of  the  complexes,  in  order  to  go  at 
the  problem  of  regulating  the  life  of  the  patient,  not  only  intelligently 
but  with  any  possibility  of  accomplishing  anything.  To  approach 
the  problem  in  any  other  way  is  to  approach  it  blindly. 

In  the  old  cases  of  precox  that  have  become  considerably  dilapidated, 
that  are  relatively  quite  inaccessible,  it  would  seem  that  the  best 
method  of  approach  was  through  the  agency  of  industrial  training. 
If  an  effort  be  made  in  this  direction  intelligently  after  a  sufficiently 
careful  analysis,  so  far  as  possible,  of  the  particular  condition  so  that  it 
will  appear  what  is  the  best  method  of  approach  in  order  to  arouse  the 
interest  and  fix  the  attention  of  the  patient,  a  great  deal  can  be  accom- 
plished in  making  this  class  of  patients  generally  useful  about  the 
hospital  and  in  limiting  their  destructive  and  filthy  tendencies  and 
adapting  them  to  a  much  healthier  series  of  adjustments  than  if  they 
are  merely  left  to  themselves. 

The  Treatment  of  the  Social  Relations. — The  endeavor  to  modify 
the  environment  of  the  patient  must  be  guided  by  what  has  been 
found  as  the  result  of  psychoanalysis.  The  precox  splitting  goes  back 
to  the  early  infantile  situations,  the  time  when  the  love  of  the  child 


710  DEMENTIA   PRECOX  GROUP 

was  given  out  in  its  entirety  to  the  immediate  members  of  the  house- 
hold, father  and  mother,  brothers  and  sisters.  Later  on  if  the  child  is 
to  become  an  efficient  adult  he  must  emancipate  himself  from  the 
thraldom  of  this  affection.  This,  however,  is  what  the  precox  has 
not  been  able  to  do,  and  the  affection  which  binds  him  to  his  infantile 
loves  is  quite  truly  felt  as  a  destructive  force  that  prevents  his  onward 
progress  in  the  world.  There  frequently  results,  therefore,  all  sorts 
of  antagonisms  addressed  to  the  members  of  the  immediate  household 
which  are  variously  expressed  and  variously  symbolized  and  distorted. 
The  love  of  the  little  child  for  the  parent  or  the  brother  or  the  sister, 
when  it  breaks  through  from  the  unconscious  into  the  conscious  life 
of  the  adult,  is  not  understood  at  its  true  value,  but  becomes  a  hateful 
thing,  and  so  characteristically  there  arise  all  sorts  of  incest  phan- 
tasies. Recently  one  such  patient  in  the  hospital  struck  and  stunned 
another  patient  and  when  called  to  task  about  it  he  instantly  protected 
himself  by  the  statement  that  "they"  were  accusing  him  of  incestuous 
relations  with  his  sister.  These  are  the  commonest  of  ideas  among 
precox  cases.  Psychoanalysis  will  orient  the  physician  with  relation 
to  these  ideas  and  enable  him  to  adjust  the  patient  intelligently. 
Such  ideas  frequently  require  the  removal  of  the  patient  to  an 
institution. 

Although  a  wholly  pessimistic  attitude  is  not  warranted  in  approach- 
ing the  problem  of  precox  in  an  individual  case,  still  it  must  be  realized 
that  after  all  one  can  hardly  expect  a  complete  recovery.  One  can 
only  hope  to  readjust  the  situation  so  that  the  patient  may  get  along 
comfortably  and  perhaps  lead  a  useful  life,  probably  upon  a  slightly 
lower  level.  The  tendency  of  the  disease  is  essentially  to  limit  the 
individual  in  the  mental  sphere,  and  this  limitation  means  of  necessity 
that  life  has  to  be  led  at  a  correspondingly  lower  and  simpler  plane 
of  adjustment.  This  is  exactly  what  the  institution  provides  for  the 
patient,  but  there  is  no  reason,  in  the  absence  of  dangerous  tendencies 
and  if  it  is  desirable  from  other  standpoints,  why  an  attempt  should 
not  be  made  in  this  direction  outside  of  an  institution.  We  know  this 
can  be  done,  for  we  see  not  infrequently  patients  getting  along  very 
well  under  the  solicitous  care  of  some  relative,  for  example,  and  when 
this  relative  dies  and  the  care  is  removed  and  their  world  is  no  longer 
carefully  arranged  for  them,  then  they  find  their  way  into  the  hospital. 

A  careful  dealing  with  all  of  the  conditions  surrounding  the  patient, 
more  particularly  dealing  with  them  intelligently  as  the  result  of  such 
psychoanalysis  as  can  be  made,  will  enable  the  physician,  in  a  very 
considerable  proportion  of  cases,  if  the  means  are  at  his  disposal, 
which  of  course  they  frequently  are  not,  to  so  adjust  the  situation 
as  to  bring  about  a  state  of  relative  calm  and  quiescence,  with  the 
preservation  of  a  considerable  degree  of  efficiency. 

Prophylaxis. — The  prophylaxis  of  dementia  precox  is  a  most  difficult 
problem,  and  in  the  first  instance  of  course  should  be  met  from  the 
eugenic  standpoint.     Marriage  should  be  very  carefully  supervised 


PROPHYLAXIS  711 

where  the  individual  comes  from  badly  tainted  stock.  Such  general 
principles  may  be  borne  in  mind,  as  for  example,  the  liability  to  mental 
disease  in  children  from  tainted  stock  is  greatest  among  the  earlier 
born  and  falls  off  rapidly,  as  Heron  has  shown,  particularly  after  the 
fourth  child,  while  Mott  in  working  out  his  Law  of  Anticipation  has 
shown  that  if  the  individual  passes  the  twenty-fifth  year  the  liability 
to  a  mental  break-down  is  very  materially  lessened. 

The  possibilities  of  prophylaxis  before  the  outbreak  of  the  psychosis 
are  not  known,  yet  it  would  seem  that  it  would  be  rational  to  endeavor 
to  deal  with  those  character  anomalies  that  we  know  favor  this  type 
of  disorder.  The  method  of  approach  will  of  course  resolve  itself 
into  an  attempt  to  define  the  lines  along  which  frank,  open  reactions 
do  not  seem  possible  to  the  individual,  particularly  along  lines  of 
definite  sex  conflicts.  In  this  particular  the  whole  matter  of  sexual 
education  has  to  be  gone  over  and  its  value  as  a  prophylactic  measure 
determined.^ 

1  See  Jelliffe,  Predementia  Precox,  Am.  Jour.  Med.  Sci.,  August,  1907,  for  a  study  of 
these  early  features  which  antedate  the  appearance  of  the  disease,  also  chapters  on  the 
treatment  of  Paranoid  States  and  Dementia  Precox,  by  Meyer  and  Campbell,  Modern 
Treatment  of  Nervous  and  Mental  Diseases,  White  and  Jelliffe,  vol.  i. 


CHAPTER  XXL 
INFECTION-EXHAUSTION  PSYCHOSES. 

This  group  of  infection-exhaustion  psychoses  is  somewhat  of  a 
heterogeneous  group,  including  all  of  the  mental  disturbances  depend- 
ent upon  the  various  infections,  as  well  as  certain  conditions  which  are 
tentatively  supposed  to  be  dependent  upon  exhaustion  and  which 
give  similar  clinical  pictures.  For  the  most  part  it  contains  the 
psychoses  dependent  upon  all  the  febrile  diseases,  for  up  to  the  present 
time  at  least  these  different  diseases  cannot  be  differentiated  solely 
from  the  mental  side. 

In  connection  with  the  similarity  of  the  manifestations  in  this 
group,  although  the  ultimate  etiological  factors  are  widely  different, 
it  is  worth  while  to  bear  a  few  considerations  in  mind.  In  the  first 
place  it  has  been  suggested  that  after  the  manner  of  thinking  regarding 
the  syphilitic  manifestations  and  the  alcoholic  psychoses,  as  already 
outlined,  that  the  mental  picture  is  not  dependent  upon  the  immediate 
infecting  agent  or  upon  the  toxin  directly  elaborated  by  the  infecting 
microorganism,  but  on  the  contrary  is  due  to  a  general  disturbance 
in  the  metabolism,  the  result  of  the  infection;  in  other  words,  that 
there  is  an  intermediate  agency  at  work,  a  metatoxin.  On  the 
other  hand  it  has  been  suggested  that  the  gamut  of  symptomatology 
which  the  neuron  may  produce  in  the  process  of  its  destruction  is 
necessarily  confined  within  certain  relatively  narrow  limits  and  that 
therefore  inimical  agencies  that  act  at  the  biochemical  level  can  only 
produce  relatively  few  groups  of  symptom-complexes.  This  is  un- 
doubtedly true.  On  the  other  hand,  if  the  individual  cases  are  care- 
fully studied  it  will  be  found  that  the  general  course  of  the  malady  and 
the  mechanisms  involved  are  quite  similar  in  the  different  cases;  in 
other  words,  that  the  patients  fall  into  one  of  very  few  groups;  still 
the  content  of  the  delirious  or  delusional  experiences  and  the  minor 
variations  in  the  manifestations  in  the  malady  must  have  another 
explanation.  This  explanation  is  naturally  the  make-up  of  the  indi- 
vidual. A  destructive  agency  at  work  in  tearing  down  can  only  tear 
down  what  has  before  been  built  up.  It  is  constrained,  in  other 
words,  to  deal  with  the  material  at  hand,  and  therefore  personal 
variations  must  be  expected;  for  example,  the  delirious  patient  will 
weave  expressions  into  his  delirious  productions  that  refer  directly  to 
his  experiences. 

Prefebrile,  Febrile,  and  Postfebrile  Psychoses. — The  same  thing 
may  be  said  regarding  fever  and  infection  which  has  already  been 
said  regarding  alcohol,  namely,  that  it  is  a  measure  of  the  mental 
stability  of  the  individual.     While  some  persons  may  remain  mentally 


PREFEBRILE,  FEBRILE,   AND  POSTFEBRILE  PSYCHOSES     713 

clear  with  a  temperature  of  a  106°  F.,  others  may  become  dehrioiis  with 
hardly  more  than  a  degree  of  temperature.  This  difference  is  a  well- 
known  one,  and  in  a  disease  like  typhoid  fever,  for  example,  the  general 
consensus  of  opinion  is  that  the  prognosis  is  most  serious  in  those  cases 
that  become  early  delirious  and  show  from  the  beginning  marked  and 
exaggerated  nervous  symptoms. 

Infection  and  Initial  Delirium. — ^Under  this  head  are  included  the 
mental  disturbances  which  develop  early  in  the  infectious  diseases 
before  there  has  been  any  rise  in  temperature.  In  fact  the  delirium 
in  these  cases  may  disappear  when  the  fever  is  fully  developed,  although 
this  is  not  the  rule,  the  usual  course  being  for  the  delirium  of  this 
period  to  go  over  into  a  fever  delirium.  This  infection  delirium  is 
also  found  in  conditions  which  are  essentially  afebrile,  as  for  example, 
rabies,  and  is  there  due  of  course  to  an  overwhelming  of  the  body 
with  toxins,  or  perhaps  to  a  bacteriemia.  This  type  of  mental  dis- 
turbance occurs  characteristically  with  the  onset  of  typhus,  in  the 
period  previous  to  the  eruption  in  smallpox,  and  has  been  observed 
in  connection  with  influenza,  acute  chorea,  especially  the  chorea  of 
pregnancy,  and  in  malaria. 

Symptoms. — The  symptoms  of  infection  delirium  are  the  usual 
symptoms  of  delirium,  which  may  present  any  degree  of  severity,  from 
mild  confusion  to  delirium  acutum,  or  collapse  delirium,  ending  in 
death.  The  diagnosis  of  initial  delirium  previous  to  the  appearance 
of  the  typical  signs  of  the  disease  of  which  it  is  a  symptom  is  of  course 
practically  impossible. 

Fever  Delirium. — Fever  delirium  is  the  psychosis  which  accompanies 
febrile  movement  and  which  in  general  varies  in  severity  hand  in  hand 
with  the  variation  in  the  severity  of  the  fever. 

It  may  be  described  in  four  stages  according  to  the  degree  of  its 
severity:  In  the  first  stage  headache,  irritability,  sensitiveness  to 
noises  and  light,  restlessness,  and  disturbing  dreams;  in  the  second 
stage  hallucinations  appear,  especially  in  the  visual  field;  the  hallucin- 
ations are  of  a  dream-like  character,  and  the  patient  may  still  be  made 
to  react  clearly;  in  the  third  stage  the  motor  disturbance  is  greater 
and  takes  on  the  character  of  jactitation;  in  the  fourth  stage  there  is 
profound  dulling  of  consciousness,  uncertain  and  ataxic  movements, 
ending  in  coma  and  death.  Of  course  this  regular  progress  of  the  delir- 
ium may  be  interrupted  at  any  point  by  an  improvement  in  the 
symptoms  and  recovery. 

The  onset  and  the  severity  of  the  delirium,  as  already  set  forth, 
shows  to  an  extent  the  mental  stability  of  the  patient.  The  delirium 
develops  or  becomes  severe  much  more  readily  in  the  unstable  and 
poorly  organized  than  in  the  stable.  The  course  of  the  delirium  may 
be  interrupted  by  an  acute  excitement  followed  by  a  stuporous  con- 
dition, which  Bonhoeffer^  describes  as  an  epileptiform  excitement,  which 

1  Die  symptomatischen  Psychosen. 


714  INFECTION-EXHAUSTION  PSYCHOSES 

is  followed  by  a  dream  state,  for  which  latter  he  retains  the  name  of 
Ziehen,  infectious  dream  state.  With  these  conditions  we  may  have 
confusing  symptoms  which  makes  the  diagnosis  diflBcult  for  a  time. 
Thus,  along  with  the  disorientation  there  may  be  flexibilitas  cerea, 
confabulation,  perseveration. 

In  certain  cases  the  orientation  is  less  disturbed,  while  the  hallucina- 
tions are  more  prominent,  and  there  is  an  outward  semblance  of  an 
hallucinosis. 

Here,  as  elsewhere  in  psychiatry,  the  various  forms  of  the  psychosis 
are  frequently  designated  by  the  prevailing  symptoms.  Thus,  the 
symptoms  found  may  be  epileptiform  excitement,  dream  states,  stu- 
porous conditions,  hallucinosis,  catatonic  and  confusional  states,  and 
the  delirium  may  be  designated  by  using  any  one  of  these  descriptive 
terms. 

Postfebrile  Psychoses. — These  conditions  either  develop  as  a  result  of 
the  passing  over  of  the  delirium  of  the  febrile  state  into  the  period  of 
convalescence,  or  they  may  take  their  origin  from  the  first,  during  the 
postfebrile  period.  In  the  latter  case  they  would  be  considered  as 
belonging  more  properly  to  the  exhaustion  psychoses,  and  yet  it  must 
be  understood  that  the  term  exhaustion  is  a  very  vague  one  and  that 
in  all  probability  it  implies  at  least  the  accumulation  of  toxins. 

The  characteristic  picture  of  this  psychosis  is  associated  with  the 
great  physical  debility  which  follows  the  subsidence  of  the  fever  in  an 
infectious  disease.  The  patient  is  weak,  tremulous,  exhausted,  and 
complains  of  being  tired.  The  mental  state  is  fundamentally  one  of 
weakness.  There  is  no  confusion,  but  the  capacity  for  attention  is 
very  much  reduced,  and  in  that  way  there  come  about  apparent  mem- 
ory disturbances.  The  patient  is  unobservant  of  his  surroundings, 
usually  somewhat  depressed,  and  may  be  very  much  occupied  with  his 
own  bodily  feelings  to  the  extent  of  having  hypochondriacal  ideas,  and 
fleeting  hallucinations  are  not  infrequent.  In  more  severe  cases  there 
may  be  more  evidence  of  mild  delirium  or  confusion,  the  mood  may  be 
more  definitely  anxious  and  fearful  and  there  may  be  delusions  char- 
acteristically of  the  persecutory  type.  The  patient  is  apt  to  be  irritable, 
cross,  and  complaining.  Occasionally  there  may  be  considerable 
motor  excitement  of  an  epileptiform  character,  and  sometimes  actual 
delirious  dream  states.  Occasionally  the  picture  of  a  mild  expansive- 
ness  is  found.  A  certain  number  of  cases  present  a  well-marked 
Korsakow  syndrome. 

Exhaustion  Psychoses. — The  term  exhaustion  in  this  connection  is 
not  intended  to  be  used  in  other  than  a  tentative  sense.  The  whole 
question  of  fatigue,  except  with  reference  to  very  specific  problems, 
such  for  example  as  muscle  fatigue,  is  still  little  understood.  In  gen- 
eral, however,  it  may  be  said  that  there  are  two  factors  in  the  problem, 
the  negative  and  the  positive.  The  former  is  the  result  of  the  actual 
wearing  out  of  a  substance  in  the  body,  as  for  example  muscle,  while 
the  second  is  the  result  of  the  formation  of  certain  poisonous  substances 


EXHAUSTION  PSYCHOSES  715 

which  result  from  the  breaking  down  of  tissue.  In  the  conditions 
which  ordinarily  are  observed  it  would  seem  evident  that  both  of 
these  factors,  the  positive  and  the  negative,  are  in  evidence,  and 
exhaustion  is  considered  where  they  appear  to  be  the  predominating 
factors.  For  example,  exhaustion  is  spoken  of  when  coming  on  acutely 
as  the  result  of  a  sudden  loss  of  a  considerable  quantity  of  blood,  or 
coming  on  more  slowly  as  a  result  of  the  debilitating  effects  of  a  chronic 
disease  such  as  carcinoma.  In  this  latter  case,  however,  it  will  be 
easily  seen  that  in  all  probability  the  toxic  element  must  enter.  Simi- 
larly in  convalescence  from  acute  illnesses  where  the  fever  has  been 
very  high  and  the  illness  has  been  prolonged  we  see  conditions  accom- 
panied by  great  physical  prostration  in  which  we  feel  that  it  is  fair  to 
assume  that  the  element  of  exhaustion  very  largely  is  responsible  for 
the  mental  picture.  Under  the  head  of  exhaustion  psychoses  two  main 
types  will  be  described — collapse  delirium  and  acute  hallucinatory 
confusion  (amentia) — but  it  must  be  understood  that  these  two  psy- 
choses are  not  necessarily  peculiar  but  only  conditions  in  which  the 
exhaustion  element  appears  to  predominate.  Either  or  both  of  them 
may  be  found  during  the  period  of  acute  infection  in  the  febrile  diseases, 
and  conditions  that  begin  with  infection  and  fever  and  give  the  picture 
of  an  infection  or  of  a  fever  delirium,  may  go  over  into  the  severer  type 
as  the  patient's  general  condition  becomes  worse  and  the  organism  is 
overwhelmed  with  the  poisoning. 

Collapse  Delirium. — This  is  the  delirium  grave,  or  the  acute  delirious 
mania  of  the  older  authors. 

The  disease  may  present  a  prodromal  period  of  restlessness,  irri- 
tability, and  insomnia,  after  which  a  condition  of  mild  confusion 
may  develop  with  only  a  slight  degree  of  perplexity  and  perhaps  fleet- 
ing hallucinations,  slight  clouding  of  consciousness,  disorientation  and 
dreamy  delusions ;  psychomotor  excitement  is  common  at  this  time, 
the  patient  being  active  and  perhaps  inclined  to  actual  violence  and 
destructiveness.  Often  associated  in  the  early  symptoms  are  accesses 
of  anxiety,  amounting  at  times  to  actual  terror.  This  condition 
gradually  becomes  worse,  and  finally  the  degree  of  excitement  becomes 
very  great,  exceeding  anything  that  we  usually  see  in  the  other 
psychoses.  When  this  extreme  form  of  excitement  is  in  evidence  the 
outcome  is  usually  apt  to  be  serious,  and  the  older  writers  believed 
it  to  be  uniformly  fatal. 

In  these  severe  cases  the  incoherence  becomes  absolute,  the  disori- 
entation complete,  the  clouding  of  consciousness  profound,  the  tem- 
perature generally  runs  high,  perhaps  as  high  as  106°  F.,  gastro-intesti- 
nal  symptoms  are  common,  there  is  usually  almost  complete  anorexia, 
coated  tongue,  offensive  diarrhea,  a  high  grade  of  indicanuria,  and 
rapid  emaciation  which  results  in  a  high  grade  of  exhaustion,  with 
typhoid  symptoms.  There  may  be  a  certain  amount  of  catalepsy, 
with  stereotyped  movements,  grimacing,  and  echolalia,  and  stuporous 
conditions;  coma,  and  death  not  infrequently  result. 


716  INFECTION-EXHAUSTION  PSYCHOSES 

This  condition  occurs  with  all  degrees  of  severity,  and  while  the  most 
severe  cases  die,  the  milder  ones  make  good  recoveries. 

It  may  often  be  quite  impossible  to  make  a  diagnosis  of  the  physical 
condition  of  the  patient  while  in  their  excitement.  It  must  be  remem- 
bered, especially  if  the  patient  has  temperature,  that  these  conditions 
are  pretty  apt  to  be  dependent  upon  some  acute  physical  illness, 
particularly  an  infection,  and  that  not  infrequently  a  deep-seated 
pneumonia,  concealed  from  the  usual  approach  by  percussion,  and 
auscultation,  is  at  the  basis  of  the  difficulty,  while  an  infection  such  as 
grip,  rheumatism,  or  the  like  may  also  be  etiological  factors. 

That  the  element  of  exhaustion  is  after  all  not  the  only  element, 
and  perhaps  not  the  most  important  element,  will  be  appreciated  if 
one  stops  to  consider  that  the  great  majority  of  patients  do  not  react 
in  this  exaggerated  way  to  acute  toxemias  or  infections.  In  all  proba- 
bility the  fundamental  factor  at  the  bottom  of  such  a  reaction  is  the 
individual  make-up. 

Acute  Hallucinatory  Confusion  (Amentia). — This  psychosis  is  less 
acute  in  its  characteristics  than  the  former.  The  symptoms  are  those 
of  a  mild  confusion  with  incoherence  and  a  considerable  degree  of 
perplexity.  There  are  usually  fleeting  hallucinations  in  the  various 
sensory  areas,  sometimes  delusions,  which,  however,  are  not  char- 
acteristically fixed,  with  a  more  or  less  changeable  emotional  attitude 
varying  with  the  content  of  the  delusions.  The  patient  is  character- 
istically in  a  condition  of  mild  motor  unrest. 

The  duration  of  the  illness  is  relatively  long.  It  may  be  prolonged 
over  several  weeks,  is  usually  from  one  to  three  months  in  duration,  and 
may  be  considerably  longer.  The  course  of  the  disease  is  not  infre- 
quently interrupted  by  lucid  intervals,  during  which  the  patient  is 
quite  clear.  These  may  last  anywhere  from  a  few  minutes  to  a  day 
or  two,  and  then  the  patient  will  lapse  back  into  his  previous  condition 
of  confusion.    This  is  an  important  point  to  bear  in  mind. 

A  very  marked  degree  of  perplexity  is  rather  characteristic  with  these 
patients.  There  is  considerable  disturbance  in  their  perception  of 
their  environment  which  they  do  not  seem  to  understand,  things 
about  them  appear  to  be  changed,  they  appear  to  be  in  some  strange 
place,  things  are  not  right,  they  do  not  understand  the  meanings  of 
things,  they  get  mixed  up,  get  into  the  wrong  bed,  and  act  in  similarly 
stupid  ways. 

Bonhoeffer^  describes  hallucinatory  and  psychomotor  catatonic 
forms,  in  the  latter  of  which  flight  of  ideas  and  incoherence  predomi- 
nate. 

In  addition  to  the  previously  described  psychoses,  Bonhoeffer^ 
describes,  as  occurring  late  in  the  course  of  the  infectious  diseases, 
and  therefore  it  would  seem  dependent  to  a  certain  extent  at  least 
upon  exhaustion,  a  hyperestheiic  emotional  state  of  mental  weakness, 

1  Op.    cit.  2  Op.  cit. 


EXHAUSTION  PSYCHOSES  717 

associated  with  physical  symptoms,  such  as  severe  headache,  paras- 
thesias,  and  pains  in  the  joints,  great  prostration,  oversensitiveness 
to  noise  and  light,  easily  frightened,  troublous  dreams.  He  also 
describes  an  amnesic  variety  resembling  Korsakow's  psychosis.  Acute 
delirium  may  also  develop  and  one  may  see  a  meningitic  form  with 
very  severe  delirious  reaction. 

The  possibilities  of  local  injuries  to  the  brain,  such  as  brain  abscess 
and  local  meningitis  must  be  held  in  mind.  When  these  develop  in 
children  they  not  infrequently  leave  sequelae,  such  as  mental  defect  and 
epilepsy. 

Treatment. — The  treatment  of  all  of  these  conditions  is,  of  course, 
in  the  main  the  treatment  of  the  underlying  disease.  In  general, 
however,  we  may  say  that  for  the  excitement  the  continuous  bath  or 
the  wet  pack,  with  perhaps  the  occasional  exhibition  of  a  hypnotic 
should  be  chosen,  rather  than  the  constant  drugging  of  the  patient,  so 
frequently  employed. 

Where  there  is  marked  lack  of  desire  for  food  and  the  condition  is 
serious  and  approaching  one  of  profound  exhaustion  there  should  be 
no  delay  in  resorting  to  artificial  feeding.  The  rectum,  of  course,  may 
be  used  if  the  stomach  is  very  irritable,  but  should  be  a  last  resort. 
It  is  preferable  to  give  small  quantities  by  the  stomach  frequently, 
and  endeavor  in  that  way  to  deal  with  the  situation,  than  to  give  rectal 
feeding,  with  the  usual  result  of  being  satisfied  with  the  giving  of  an 
amount  of  food  which  is  really  very  inadequate. 

In  the  extreme  exhaustion  of  the  later  stages,  especially  where  there 
is  marked  dehydration,  hypodermoclysis  often  gives  most  excellent 
results. 

Typhoid  Fever.- — An  initial  delirium  in  typhoid  fever  is  generally 
conceded  to  be  of  most  ominous  import,  while  an  early  delirium  which 
is  continuous  and  severe  is  also  of  bad  prognostic  significance.  With 
this  disease  the  good  effects  of  the  bath  treatment,  as  used  by  the 
Brand  method,  is  especially  well  seen.  Cold  baths  for  the  reduction  of 
temperature  have  as  one  of  their  most  important  results  their  sedative 
and  calming  effect  upon  the  nervous  symptoms. 

The  possibility,  in  all  such  diseases  as  this,  of  the  localization  of  the 
infection  in  the  meninges  should  be  thought  of,  and  in  cases  of  marked 
delirium,  therefore  the  possibility  of  a  meningitis  should  be  borne  in 
mind.  Lumbar  puncture  may  be  valuable  under  these  circumstances, 
both  for  diagnostic  purposes  and  for  the  relief  of  pressure. 

In  the  late  stages,  during  convalescence,  special  efforts  should  be 
made  to  make  the  dietary  as  full  and  liberal  as  possible. 

Erysipelas,  the  various  exanthems,  and  the  several  types  of  malaria 
are  also  not  infrequently  complicated  by  mental  symptoms,  while 
grippe  often  produces  profound  depressions  associated  with  great 
physical  exhaustion  hanging  over  during  a  long  convalescence.  We 
must  remember  in  all  of  these  conditions,  particularly  in  the  exanthems, 


718  INFECTION-EXHAUSTION  PSYCHOSES 

the  possibility  of  meningeal  focalization.  Grippe  sometimes  also 
produces  meningeal  inflammation. 

Acute  articular  rheumatism  is  especially  important  in  this  connec- 
tion because  of  its  relation  to  acute  chorea  and  the  so-called  chorea 
insaniens. 

Pneumonia  is  frequently  associated  with  mental  symptoms.  Very 
many  of  the  cases  of  delirium  tremens  owe  their  severity  to  a  pneu- 
monia, usually  a  masked  form  of  this  disease,  either  a  central 
pneumonia  or  one  located  in  the  upper  lobes,  and  therefore  not  so 
readily  diagnosed. 

In  connection  with  all  this  class  of  diseases  it  must  be  borne  in  mind 
the  many  complications  which  may  arise  in  their  course  and  upon 
which  the  mental  symptoms  may  depend  other  than  the  meningitides, 
as  for  example,  the  acute  types  of  nephritis  in  connection  with  scarlet 
fever,  the  middle-ear  complications  of  measles,  and  the  endocardial 
complications  of  rheumatism.  Not  infrequently,  too,  these  various 
complications  may  depend  upon  mixed  infections. 


CHAPTER  XXII. 
THE  TOXIC  PSYCHOSES. 

Alcoholism. — It  is  generally  conceded  that  alcohol  is  a  powerful 
poison  and  as  such  if  taken  in  large  quantities  or  over  a  long  period 
of  time  produces  serious  damage  to  the  individual.  There  is,  unfor- 
tunately, associated  with  this  view  the  opposite  view  that  alcohol  is  a 
valuable  medicine  in  certain  conditions,  particularly  that  it  is  a  valuable 
stimulant,  while  among  certain  peoples  it  is  generally  accepted  as  an 
essential  article  in  the  daily  dietary. 

As  a  matter  of  fact  the  toxic  properties  of  alcohol  far  outweigh 
any  possible  beneficent  effects  that  it  may  have.  In  fact  it  is  question- 
able whether  alcohol  should  be  considered  in  any  other  sense  than  as  a 
poison.  It  has  no  special  medicinal  properties  that  are  of  value,  it  is 
not  a  stimulant,  and  at  most  might  be  considered  as  a  hypnotic, 
especially  in  old  people  with  some  arteriosclerosis.  Other  drugs 
can  be  used,  however,  quite  as  well  and  more  safely.  It  is  probable 
that  the  widespread  belief  in  its  efficacy  for  all  sorts  of  conditions  is 
based  upon  an  effort  at  the  justification  for  its  use. 

The  part  that  alcohol  plays  in  the  production  of  mental  disorders 
is  extremely  difficult  to  express  in  definite  terms.  Perhaps  12  to  15 
per  cent,  of  the  psychoses  are  dependent  upon  alcohol  as  the  principal 
etiological  factor,  and  yet  any  such  figure  as  this  is  extremely  mislead- 
ing, for  we  really  do  not  know  how  alcohol  brings  about  its  results, 
and  especially  it  is  not  known  whether  it  is  primarily  the  alcohol 
which  is  to  blame  or  whether  secondarily  the  metabolism  disturbances 
which  are  produced  by  its  continuous  use.  It  is  of  the  highest  sig- 
nificance that  of  the  cases  that  come  to  autopsy  a  very  considerable 
number  of  them  in  the  general  hospitals  and  poor  houses  are  found  to 
suffer  from  cirrhosis  of  the  liver,  while  in  the  hospitals  for  the  insane 
this  condition  is  of  rare  occurrence.  The  indication  is  very  clear  that 
the  psychoses  are  dependent  upon  some  peculiarity  of  make-up  of  the 
individual  which  is  affected  in  an  exaggerated  way  by  alcohol  or  of 
which  alcoholism  is  the  expression.  If  this  is  true,  then  the  alcoholism 
is  only  a  surface  indication,  and  the  true  etiological  factors  lie  deeper. 

Psychology. — There  are  many  types  of  persons  who  drink  and  there 
are  many  reasons  for  drinking.  The  usual  reasons  given  refer  to  social 
conditions.  We  are  rather  taught  to  believe  that  the  social  demands 
are  of  such  a  nature  as  to  require  a  certain  amount  of  drinking,  and  that 
this  drinking  once  started  tends  to  perpetuate  itself  and  to  finally 
become  a  fixed  habit.  This  way  of  looking  at  the  situation,  like  the 
belief  in  the  beneficent  effect  of  alcohol,  is  very  largely  at  least  an  effort 


720  THE  TOXIC  PSYCHOSES 

at  justification.  The  social  conditions  cannot  be  changed.  We  have 
to  submit  to  them,  and  therefore  the  drinking  is  inevitable  and  not  the 
fault  of  the  individual.  This  really  offers  no  adequate  explanation, 
and  in  all  probability  there  is  very  little  truth  in  the  statement.  People 
do  not  drink  simply  and  solely  because  they  have  acquired  a  habit 
of  drinking.  The  habit  element  is  the  least  important  in  the  whole 
situation,  and  if  that  were  the  only  thing  to  be  dealt  with  the  problem 
of  alcoholism  and  of  other  habituations  would  be  relatively  simple. 
People  drink  because  of  definite  returns  which  they  get  from  drinking. 
A  given  number  of  persons  all  placed  under  the  same  conditions,  social, 
etc.,  do  not  all  drink.  It  is  only  some  of  them  who  drink,  and  those 
who  drink  do  so  not  only  because  they  get  definite  desirable  results, 
but  because  those  results  are  practically  necessary  for  them.  In  other 
words,  far  more  important  than  the  question  of  habit  formation,  is 
the  question  of  the  individual  psychology.  Here  again,  as  has  been 
seen  already  in  dealing  with  the  psychoses,  the  question  of  mental 
conflicts  is  most  important.  When  the  individual  is  confronted  by 
situations  to  which  he  cannot  adjust  adequately,  when  the  world  of 
reality  makes  demands  which  are  too  great  for  him  to  meet,  one  of  the 
ways  in  which  the  individual  reacts  to  such  a  condition  is  by  narcotizing 
himself  and  so  withdrawing  from  the  whole  situation.  Alcohol  then 
becomes  an  agent  which  helps  the  patient  to  get  away  from  the  conflicts 
thrust  upon  him  by  reality,  it  helps  him  to  withdraw  within  himself, 
helps  him  to  live  in  the  world  of  phantasy  where  things  come  true  as  he 
wishes  them.  Under  these  circumstances  it  can  be  seen  why  what 
appears  to  be  a  habit  is  formed.  The  moment  the  individual,  harassed 
by  the  obsolutely  inacceptable  demands  of  the  world,  flnds  an  avenue 
of  escape  in  which  he  can  rest  from  their  harassings,  finds  the  possi- 
bility of  peace,  of  repose,  he  finds  it  equally  impossible  to  resist  the 
temptation  to  avail  himself  of  it  and  of  course  he  usually  continues 
to  avail  himself  of  it.  He  is  rendered  more  and  more  incapable 
of  meeting  the  conflict  efficiently.  Therefore  a  vicious  circle  is  estab- 
lished and  the  individual  is  hopelessly  involved. 

Aside  from  the  class  of  individuals  described  above,  it  must  be 
constantly  borne  in  mind  that  indulgence  in  alcohol  is  oftentimes 
the  expression  of  a  psychosis.  For  example,  the  recurrent  attacks  of 
manic-depressive  psychosis  may  be  ushered  in  by  alcoholic  indulgence, 
and  if  one  is  not  keenly  observant  he  may  easily  suspect  that  he  is  dealing 
with  an  alcoholic  psychosis  rather  than  with  a  manic-depressive.  The 
same  thing  of  course  may  be  said  of  dementia  precox  which  is  often 
found  associated  with  alcohol,  and  with  paresis,  which  not  infrequently 
has  a  history  of  alcoholism  in  its  early  stages.  Then  it  must  be 
remembered  that  in  certain  post-traumatic  conditions,  that  is,  cases 
following  especially  head  injury  and  sunstroke,  and  in  arteriosclerotic 
and  senile  conditions  the  patient  may  react  in  an  exaggerated  way  to 
alcohol  and  that  very  small  doses  may  produce  very  pronounced 
effects.    This  is  true  also  in  connection  with  certain  other  conditions, 


ALCOHOLISM  721 

particularly  imbecility  and  epilepsy.  Here  pronounced  reactions  to 
alcohol  are  found,  and  it  is  important  to  evaluate  the  importance  of 
the  alcohol  in  the  entire  situation. 

Finally,  there  are  certain  psychoses  which  appear  to  be  essentially 
alcoholic;  to  depend  upon  prolonged  indulgence  in  alcohol.  Of  the 
acute  conditions  due  to  alcohol  drunkenness  is  the  most  typical, 
while  of  the  chronic  conditions  dependent  upon  alcohol,  delirium 
tremens,  alcoholic  hallucinosis,  and  Korsakow's  psychosis  are  the 
most  important.  These  latter  because  they  appear  to  be  dependent 
upon  something  other  than  simple  alcohol,  as  they  never  occur  as  the 
result  of  single  large  doses,  but  can  only  occur  in  a  person  chronically 
addicted  it  has  been  proposed  to  call,  after  the  manner  of  the  psychoses 
due  to  syphilis,  the  meta- alcoholic  psychoses.  With  this  introduction 
a  short  description  of  each  of  the  mental  pictures  dependent  directly 
or  indirectly  upon  alcohol  will  be  given. 

Drunkenness. — The  general  phenomena  of  drunkenness  are  too 
well  known  to  require  detailed  description,  except  that  perhaps  the 
less  evident  manifestations  are  not  generally  thought  of.  The  pro- 
gressive disturbance  of  coordination  of  the  motor  centers,  the  higher 
being  thrown  out  of  adjustment  first,  and  the  progressive  disturbance 
of  the  sensory  apparatus  in  the  same  way  produce  disturbances,  the 
latter  of  which  are  entirely  subjective  and  so  are  not  generally  observed. 
The  mood  of  the  drunken  man  is  variable.  Each  man  reacts  to  alcohol 
in  a  way  peculiar  to  himself,  but  in  a  general  way  there  may  be  con- 
sidered to  be  two  classes  into  which  cases  of  drunkenness  may  be 
divided,  the  exalted  and  the  depressed,  the  former  closely  resembling 
the  manic  phase  of  the  manic-depressive  in  his  extreme  loquaciousness 
and  hyperactivity,  while  in  the  latter  the  patient  withdraws  within 
himself,  being  sullen,  morose,  and  disinclined  to  associate  with  others. 

One  of  the  important  considerations  under  this  head  is  the  degree  of 
ease  with  which  the  individual  is  seen  to  react  to  alcohol.  Alcohol, 
like  fever,  may  be  used  to  express  the  measure  of  cerebral  resistance; 
unstable  and  defective  individuals  reacting  to  both  in  an  exaggerated 
way. 

Pathological  Drunkenness. — Drunkenness  that  exhibits  unusual 
features  which  lead  the  individual  to  perform  strange  acts  or  acts  of 
violence  or  which  produce  serious  physical  symptoms  are  known  as 
conditions  of  pathological  drunkenness.  Persons  in  this  condition 
may  develop  well-marked  hallucinations  or  delusions  to  which  they 
tend  to  react,  while  convulsive  attacks  are  not  infrequent,  and  the 
whole  period  is  often  obliterated  from  the  memory  by  an  amnesia. 

Chronic  Alcoholism. — The  effects  of  alcoholic  poison  may  be  ex- 
exliibited  in  any  organ  of  the  body,  more  particularly  the  central 
nervous  system,  the  stomach,  the  liver,  the  kidneys,  and  the  blood- 
vessels. The  effects  on  the  nervous  system  are  shown  in  various 
disturbances  of  sensation,  such  as  the  paresthesias,  amblyopia,  amau- 
rosis, dulness  of  hearing,  of  touch,  etc.,  while  in  the  motor  realm 
46 


722  TEE   TOXIC  PSYCHOSES 

we  find  tremor  and  epileptiform  attacks,  with  general  motor  en- 
feeblement  characteristically.  The  mental  changes  are  gradual  and 
progressive,  the  intelligence  is  blunted,  the  judgment  is  impaired, 
the  moral  sense  dulled,  while  actual  delusions  not  infrequently  develop. 

While  all  of  these  changes  may  occur  in  chronic  alcoholism,  it  is 
usual  to  see  in  individual  cases  one  organ  more  especially  selected  out 
by  the  alcohol  for  its  destructive  effects.  With  the  beer  drinkers 
who  absorb  several  liters  of  beer  each  day,  cardiac  hypertrophy  is 
quite  common.  Some  patients  develop  serious  kidney  complications, 
while  others  are  able  to  drink  over  long  periods  of  years  without  any 
material  impairment  of  the  kidneys.  The  same  remark  applies  to  the 
liver,  while  the  effects  upon  the  nervous  system  and  the  bloodvessels 
are  equally  varied.  It  is  characteristic,  too,  that  the  individual  as  a 
whole  varies  in  the  destructive  effect  which  alcohol  has  upon  him, 
some  individuals  apparently  being  able  to  use  large  quantities  over 
considerable  periods  of  time  without  noticeable  impairment.  The 
general  outward  appearance  of  health,  however,  which  many  indulgers 
in  alcohol  show  is  sometimes  rudely  dissipated  when  they  are  attacked 
by  an  acute  illness,  for  however  well  they  may  have  been  able  to  go 
on  with  the  routine  day's  work,  their  weakness  is  shown  when  they  are 
subjected  to  the  special  stress  of,  for  example,  a  pneumonia,  and  the 
death  rate  in  this  class  of  persons  is  much  higher  than  in  abstainers. 

Delirium  Tremens. — Delirium  tremens,  while  an  acute  manifesta- 
tion of  alcoholism,  can  only  occur  in  a  person  suffering  from  the  effects 
of  chronic  alcoholic  poisoning.  It  may  manifest  itself  sometimes  as 
the  result  of  a  prolonged  debauch,  sometimes  as  the  result  of  an  illness 
or  injury  in  a  chronic  alcoholic.  It  has  often  been  maintained,  and  is 
still  believed  by  many,  that  the  delirium  is  not  infrequently  the  result 
of  a  sudden  withdrawal  of  alcohol,  as  for  example  when  a  patient  is 
taken  to  the  hospital  for  an  injury  nothing  is  thought  of  his  alcoholic 
tendencies  until  he  develops  a  delirium.  In  these  cases  the  delirium 
is  presumed  to  be  due  to  the  fact  that  the  patient  did  not  get  his  usual 
supply  of  alcohol.  There  is  no  good  reason  for  this  opinion,  and  it 
must  be  remembered  that  in  the  prodromal  period  of  delirium  tremens 
not  infrequently  the  patient  has  experienced  a  disgust  of  liquor  for  a 
number  of  days.  A  pretty  effective  negation  of  the  theory  of  an 
abstinence  delirium  is  derived  from  the  English  prison  statistics,  which 
show  that  in  the  year  1907  there  were  63,000  inebriates  who  were 
suddenly  deprived  of  alcohol  by  confinement,  and  in  this  number  only 
246  developed  delirium  tremens,  less  than  one-half  of  one  per  cent. 

Symptoms. — The  symptoms  of  delirium  tremens  may  come  on  slowly, 
being  preceded  by  several  days  of  general  physical  upset,  with  transient 
sensory  falsifications  and  perhaps  delusional  interpretation,  which 
show  a  special  tendency  to  come  on  at  night  or  under  conditions  in 
which  accurate  perception  is  interfered  with.  On  the  other  hand, 
the  delirium  may  come  on  very  rapidly.  A  case  is  recalled  of  a  long- 
shoreman, a  man  of  perhaps  thirty-five,  a  giant  in  physique,  who  walked 


DELIRIUM   TREMENS  723 

into  the  hospital  at  noon  with  a  sprained  ankle ;  the  ankle  was  strapped, 
and  he  was  put  to  bed.  That  night  he  was  in  the  wildest  delirium, 
and  the  next  morning  he  was  dead. 

Following  these  prodromal  symptoms  the  delirium  appears  in  its 
complete  manifestations.  It  is  a  typical  toxic  delirium  with  multiform 
disorders  of  the  sensorium.  The  hallucinations  predominate  in  the 
visual  sphere,  although  tactile  hallucinations  are  very  common.  The 
patient  is  manifestly  very  sick,  he  is  greatly  depressed  physically,  he 
is  tremulous,  the  tremor  being  so  constant  as  to  have  given  the  name 
to  the  condition,  and  his  mood  is  characteristically  one  of  apprehension, 
anxiety,  and  fear.  He  is  disoriented,  mistakes  the  people  about  him ; 
not  infrequently  his  delirium  is  an  occupation  delirium  and  he  believes 
himself  back  at  his  accustomed  work,  but  it  is  characteristically  filled 
with  fearsome  sights,  and  he  is  in  constant  terror  from  the  invasion  of 
the  numerous  animals  that  he  may  see  about  him. 

In  the  somatic  realm  the  pulse  is  rapid,  the  tremor  constant,  the 
skin  bathed  in  perspiration,  the  tongue  foul,  the  appetite  nil,  and  there 
may  perhaps  be  a  marked  grade  of  albuminuria.  The  temperature  is 
characteristically  either  normal  or  below  normal,  but  it  may  be  high, 
in  which  case  we  have  what  is  known  as  the  febrile  type  of  the  disease, 
which  is  generally  fatal.  All  sorts  of  complications  may  naturally 
occur.  Acute  cardiac  dilatation  sometimes  causes  death,  while  one 
of  the  most  frequent  of  the  complications  in  severe  cases  is  "wet  brain." 
In  this  condition  the  patient  sinks  into  a  low  muttering  delirium,  the 
temperature  falls  to  subnormal,  the  face  is  pale  and  bathed  in  cold 
perspiration,  the  pupils  dilated,  there  may  be  some  rigidity  of  the  neck 
with  a  tendency  to  a  bending  back  of  the  head ;  the  patient  sinks  into  a 
comatose  condition  and  dies. 

The  psychosis  runs  an  acute  course  of  about  three  days  and  usually 
terminates  by  a  long  sleep  in  recovery.     Ten  to  15  per  cent.  die. 

Treatment. — The  treatment  of  delirium  tremens  should  be  supporting, 
liquid  concentrated  food,  predigested  if  necessary.  The  bowels  and 
the  kidneys  should  be  freely  flushed,  heart  stimulants  are  necessary 
to  prevent  cardiac  failure,  and  hypnotics  often  required  to  produce  rest. 
For  the  excitement,  hydrotherapy  in  the  form  of  the  continuous  bath, 
or  if  this  is  not  available,  cold  packs  are  preferable  to  drugs.  The 
constant  thing  to  be  kept  in  mind  is  the  support  of  the  strength  of  the 
patient,  and  the  logical  way  to  accomplish  this  end  is  by  feeding.  If 
the  patient  refuses  food  no  time  should  be  wasted.  He  should  be 
immediately  fed  with  the  tube,  preferably  as  often  as  three  times  a 
day  in  small  amounts,  watching  the  stools  and  govering  the  quantities 
given  in  each  feeding  by  the  amount  the  patient  is  able  to  digest.  If 
the  patient  is  unable  to  retain  the  food  owing  to  acute  irritability  of 
the  stomach  and  constant  vomiting,  feeding  by  the  rectum  should  be 
resorted  to,  while  if  the  prostration  is  extreme  and  there  is  emaciation 
and  the  deprivation  of  fluid  considerable,  hypodermoclysis  is  a  valuable 
adjunct. 


724  THE   TOXIC  PSYCHOSES 

Korsakow's  Psychosis. — This  psychosis  is  found  typically  in  asso- 
ciation with  alcoholic  polyneuritis,  although  the  same  mental  state 
may  be  found  with  a  polyneuritis  of  different  origin,  as  for  example  the 
metallic  poisons,  some  of  the  infections  such  as  tuberculosis  and 
influenza,  and  some  of  the  endogenous  toxins  as  in  diabetes.  The 
syndrome  occurs  also  in  connection  with  general  organic  changes  of 
the  central  nervous  system  as  in  paresis,  arteriosclerosis,  and  senility. 
In  the  latter  case  it  is  a  part  of  the  clinical  picture  of  presbyophrenia. 

While  this  psychosis  occurs  typically  in  connection  with  polyneuritis, 
the  evidences  of  a  polyneuritis  may  be  very  slight.  They  should  be  care- 
fully examined  for,  especially  by  pressure  over  the  large  nerve  trunks, 
which  will  often  elicit  painful  points,  rather  typically  at  the  points  of 
exit  through  the  bony  foramina.  The  Lasegue  sign  should  be  sought 
for. 

Korsakow's  psychosis  is  sometimes  designated  in  contradistinction 
to  delirium  tremens,  which  is  spoken  of  as  an  acute  alcoholic  delirium, 
as  a  chronic  alcoholic  delirium.  In  fact  the  attack  may  begin  with 
a  typical  delirium  tremens  which  merges  into  the  chronic  delirium  of 
Korsakow's  psychosis  instead  of  clearing  up,  although  this  is  not  the 
usual  method  of  onset.  Not  infrequently,  however,  a  history  of  pre- 
vious attacks  of  delirium  tremens  may  be  elicited. 

The  mental  symptoms  are  the  result  of  a  combination  of  attention 
and  memory  disorder.  The  memory  disorder  is  of  the  nature  of 
a  lack  of  impressibility.  The  result  of  this  combination  is  a  special 
type  of  amnesia.  There  is  defect  in  the  recording  of  current  events. 
The  patient  is  usually  disoriented,  to  some  extent  at  least,  and  the 
things  that  have  recently  happened  cannot  be  recalled.  These  defects 
of  memory  are  characteristically  supplied  by  fabrications  of  all  sorts 
of  degrees  of  probability  and  grotesqueness,  which  are  usually  related 
by  the  patient  with  a  composed  bearing  and  with  every  outward 
appearance  of  relating  facts,  or  at  least  occurrences  which  he  himself 
believes.  These  fabrications  do  not  correspond  if  the  patient  is  asked 
at  intervals  about  occurrences  covering  a  certain  period,  and  they  may 
often  be  guided  by  suggestions  from  the  questioner.  Not  infrequently 
the  patient  in  his  fabrications  invents  occurrences  which  account  for 
his  symptoms,  for  example,  a  patient  who  is  suffering  from  a  certain 
amount  of  pain  in  his  legs  as  a  result  of  his  polyneuritis,  even  though 
he  may  have  been  confined  to  his  bed  for  weeks,  will  say  that  he  was 
out  that  morning  and  climbed  a  long  hill  and  tired  his  legs  out,  and  in 
that  way  he  accounts  for  their  aching. 

The  experiences  which  these  patients  relate  are  of  a  delirious  char- 
acter and  not  infrequently  it  is  rather  difficult  to  distinguish  the 
fabrications  from  the  dreams,  and  it  would  appear  that  the  two  often 
merge  into  one  another. 

Physically  the  patient  usually  presents  the  signs  of  a  polyneuritis, 
although  these  may  be  very  mild.  When  the  neuritis  is  severe,  foot- 
drop  and  wrist-drop  are  characteristic  signs,  as  the  nerves  of  the  exten- 


ALCOHOLIC  HALLUCINOSIS  725 

sors  of  the  forearm  and  leg  are  most  characteristically  involved  in 
alcoholic  neuritis. 

The  serious  complications  of  the  disease  are  either  dependent  upon 
intercurrent  affections  such  as  pneumonia  or  upon  the  involvement  of 
important  nerves  such  as  the  vagus,  vagus  involvement  of  course  being 
pretty  apt  to  be  fatal.  A  serious  complication  is  a  neuritis  of  the 
phrenic  nerve  with  paralysis  of  the  diaphragm  on  one  or  both  sides 
with  resulting  tympanitis  and  serious  embarrassment  of  cardiac  and 
respiratory  activity. 

Pathology. — The  pathology  of  the  disease  shows  that  it  is  by  no 
means  confined  to  the  peripheral  nerves  nor  even  to  the  motor  areas 
of  the  central  nervous  system,  but  that  there  is  a  very  general  involve- 
ment of  the  entire  central  nervous  system,  the  cord,  basal  ganglia, 
and  the  cortex  all  showing  lesions,  although  there  appears  to  be  a 
tendency  to  the  focalization  of  the  pathological  process.  As  a  result 
we  get  certain  focal  symptoms  as  aphasia,  apraxia,  hemianopia,  etc. 
This  wide  distribution  of  the  pathological  disturbances  and  their  ten- 
dency to  focalization  is  due  to  the  fact  that  the  disease  involves  the 
bloodvessels.  The  smaller  vessels  proliferate  and  present  evidences 
of  endo-,  meso-  and  periarteritis  with  frequent  ruptures.  When 
this  condition  is  focalized  in  the  mid-brain  and  gives  rise  to  various 
ocular  palsies  we  have  the  syndrome  of  acute  hemorrhagic  polioencepha- 
litis of  Wernicke.  This  is  frequently  observed  in  chronic  alcoholics. 
The  condition  is  typically  ushered  in  by  severe  and  prolonged  vomiting, 
which  is  followed  by  marked  confusion  with  delirium,  vertigo,  some- 
times headache,  and  somnolence.  Optic  neuritis  may  be  present. 
Death  usually  occurs  in  from  one  to  two  weeks. 

With  a  disease  of  this  character  having  a  pathology  so  widely 
distributed  throughout  the  nervous  system  and  dependent  upon 
chronic  toxemia  we  can  understand  how  very  many  types  have  been 
described.  These  types  or  clinical  forms  are  nothing  more  than 
descriptions  of  the  disease  in  which  certain  symptoms  are  especially 
prominent,  and  so  we  have  amnesic,  confusional,  delusional,  anxious, 
demented,  delirious,  stuporous,  hallucinatory  forms  described  by 
different  authors. 

Treatment. — The  treatment  of  the  condition  is  of  coiu-se  the  with- 
drawal of  alcohol  and  the  treatment  of  a  general  toxic  state  along 
supportive  lines.  The  deformities  which  are  due  to  the  paralysis 
should  be  dealt  with  by  recognized  orthopedic  measures.  It  should  be 
remembered  that  while  the  patient  is  in  bed  and  delirious  the  weakened 
extensors  of  the  forearm  and  leg  should  not  be  stretched  by  the  weight 
of  the  limb,  but  should  be  supported,  particularly  the  weight  of  the 
bed  clothes  should  be  removed  from  the  feet. 

Alcoholic  Hallucinosis. — This  condition  is  also  an  expression  of 
chronic  alcoholism  and  may  be  preceded  by  attacks  of  delirium  tremens. 
It  is  characterized  by  hallucinations,  auditory  predominating,  in  this 
respect  strongly  contrasted  to  delirium  tremens,  and  delusions  of  a 


726  THE  TOXIC  PSYCHOSES 

persecutory  character  which  harmonize  and  are  explanatory  of  the 
hallucinations.  It  is  quite  characteristic  that  the  hallucinations  and 
the  delusions  deal  with  sexual  matters,  the  patient  frequently  being 
abused  by  "the  voices"  for  comitting  some  sexual  crime  or  is  accused  of 
sexual  perversions.  The  delusions  are  practically  invariably  persecu- 
tory, although  occasionally  expansive  elements  may  enter  the  picture. 
Not  infrequently,  too,  the  patient  is  very  much  frightened,  as  when  he 
thinks  he  is  being  closely  pressed  by  his  persecutors  who  are  determined 
upon  taking  his  life.  There  are  not  a  few  border-line  mixtures  of  this 
psychosis  and  delirium  tremens. 

The  condition  is  essentially  an  acute  paranoid  state  and  as  such 
its  explanation  is  the  same  as  the  explanation  of  paranoia.  In  other 
words,  there  is  in  these  cases  a  fixation  at  the  homosexual  level,  and 
this  accounts  for  the  very  great  frequency  of  the  sexual  character  of 
the  hallucinations  and  the  references  to  sexual  perversions.  The 
alcohol  which  is  taken  by  the  individual  because  he  cannot  deal 
efficiently  with  reality  causes  him  to  turn  back  upon  himself  and 
produces  a  reanimation  of  this  early  fixation,  and  then  the  mechanism 
of  projection  comes  in  as  a  distortion  to  make  it  appear  that  the 
suffering  has  its  origin  from  without. 

The  delusional  system  is  rapidly  systematized  so  that  in  certain 
cases  the  patient  might  give  the  impression  of  a  paranoiac.  The 
course  of  the  disease  is  usually  prolonged  over  a  number  of  weeks, 
and  not  infrequently  a  number  of  months.  It  generally  ends  in  re- 
covery, but  sometimes  merges  into  chronicity.  The  disease  is  not 
fatal  and  its  pathology  is  therefore  the  pathology  of  chronic  alco- 
holism. 

Alcoholic  Fseudoparesis. — In  a  few  patients  prolonged  alcoholic 
intoxication  produces  a  picture  closely  resembling  general  paresis. 
In  these  cases  we  find  an  expansive  delirium  combined  with  the  signs 
of  alcoholism,  such  as  ataxia,  speech  defects,  and  tremor.  It  must  be 
remembered  too  that  pupillary  anomalies  quite  regularly  occur  in 
severe  grades  of  intoxication  and  even  an  Argyll-Robertson  pupil 
has  been  observed.  These  observations  should  be  taken  with  a 
certain  amount  of  reservation  because  it  is  quite  impossible  often 
to  tell,  without  the  aid  of  instruments,  exactly  what  the  pupillary 
reaction  may  be  and  especially  whether  it  is  entirely  lost,  and  it  is 
generally  conceded  that  toxic  conditions  will  produce  a  slowing  of  the 
light  reflex.  On  the  other  hand,  it  has  been  thought  by  some  that 
where  these  marked  pupillary  disturbances  are  found  that  it  indicated 
the  presence  of  syphilis.  Therefore  these  observations  should  be 
correlated  with  the  serological  findings.  These  cases  clear  up  promptly 
upon  the  removal   of  alcohol. 

Alcoholic  Pseudoparanoia. — In  some  cases  of  chronic  alcoholism 
a  fairly  circumscribed  delusional  system  may  develop  which  character- 
istically takes  the  form  of  delusions  of  marital  infidelity.  In  endeavor- 
ing to  interpret  this  delusion  the  paranoia  mechanisms  must  be  borne  in 


OPIUM  727 

mind.  Quite  commonly,  however,  the  dehision  when  it  occurs  in  man 
is  a  defense  reaction  to  impotence,  which  has  been  Uxrgely  produced, 
probably,  by  the  alcoholic  indulgence.  Instead  of  realizing  his  own 
impotence,  which  is  an  inacceptable  thought,  he  blames  his  wife  for 
being  untrue  to  him.  These  cases  are  essentially  chronic  and  persist 
at  least  as  long  as  the  alcohol  is  indulged  in,  while  even  when  it  is 
removed  they  may  be  a  long  time  clearing  up  and  may  perhaps 
graduate  into  a  chronic  delusional  state. 

Alcoholic  Epilepsy. — In  a  chronic  alcoholic  toxemia  it  is  not  strange 
that  epileptiform  convulsions  should  occasionally  develop  and  recur 
from  time  to  time.  The  outward  characteristics  of  the  convulsion 
are  in  every  way  the  characteristics  of  an  epileptic  attack.  If  the 
person  is  not  essentially  epileptogenic  the  convulsions  will  subside 
on  the  removal  of  the  alcohol. 

Dream  States. — In  conditions  of  pathological  drunkenness  it  is 
not  infrequent  for  the  patient  to  have  no  recollection  of  the  period 
during  which  he  was  intoxicated.  If,  during  this  period,  he  has  been 
engaged  in  some  sort  of  occupation,  business  transactions,  travel,  or 
what  not,  or  perhaps  engaged  in  the  performance  of  criminal  acts, 
he  may  still  wake  up  with  no  recollection  at  all  of  this  period,  although 
during  it  he  outwardly  appeared  to  be  in  a  normal  state.  Some  people 
are  especially  liable  to  these  dream  states.  It  would  seem  that  they 
present  a  tendency  to  the  doubling  of  their  personality  and  that  the 
alcohol  helps  to  produce  these  somnambulic  episodes. 

Dipsomania. — Dipsomania  is  a  term  applied  to  a  periodical  impulse 
to  drink.  Drinking  is  only  the  outward  manifestation  of  the  trouble. 
It  is  a  recurrent  neuropathic  attack  which  demands  the  narcotizing 
results  of  alcoholic  indulgence. 

Opium. — The  habitual  use  of  opium  in  some  form  has  become 
common  and  among  all  classes  in  society.  The  same  thing  may  be 
said  with  reference  to  the  reasons  for  taking  opium  as  has  been  said 
with  reference  to  alcohol.  The  opium  habitue  is  a  person  primarily 
of  neuropathic  taint,  the  mere  opium  taking  or  the  symptoms  it 
produces  being  but  surface  indications  of  the  real  trouble. 

Symjjtoms. — The  general  symptoms  of  its  use  are  well  known.  In 
small  doses  it  is  mildly  stimulating  and  produces  a  pleasant  euphoria, 
while  if  it  is  taken  in  large  doses  and  continuously  it  leads  to  a  mental 
blunting,  a  general  feeling  of  malaise,  inability  to  make  effort,  with 
marked  physical  disturbances  such  as  tremor,  muscular  weakness, 
constipation,  paresthesias,  etc.  It  is  not  infrequent  for  opium  in  some 
of  its  forms  to  be  combined  with  addiction  to  alcohol  or  cocaine.  Con- 
tinuous use  may  simply  produce  a  thoroughly  crippled  individual  who 
is  alternately  in  a  mildly  euphoric  state  or  in  a  condition  of  stupidity, 
malaise,  and  indolence.  When  a  psychosis  develops,  it  is  character- 
istically an  hallucinated  state  usually  with  paranoid  coloring,  or  it 
may  be  distinctly  delirious.  The  prognosis  is  of  course  not  good  unless 
the  underlying  neuropathic  taint  can  be  dealt  with. 


728  THE   TOXIC  PSYCHOSES 

The  use  of  opium  has  much  less  tendency  than  that  of  alcohol  to 
produce  tissue  degeneration. 

Treatment. — ^The  treatment  is  usually  the  prompt  removal  of  the 
large  doses.  These  can  be  readily  withdrawn  until  the  patient  is 
reduced  to  the  actual  necessary  amount  of  morphin  to  get  through  the 
day  comfortably  with,  which  is  about  two  grains.  From  this  point  on 
withdrawal  should  be  gradual,  carefully  looking  after  the  symptoms 
of  abstinence  as  they  appear.  These  symptoms  refer  particularly  to 
the  heart,  the  nervous  system,  and  the  gastro-intestinal  system. 
Sometimes  profound  collapse  may  occur  with  the  withdrawal  of  the 
drug.  Cardiac  conditions  should  be  watched  and  stimulants  adminis- 
tered if  there  is  any  sign  of  weakness.  For  the  diarrhea  opium  should 
be  avoided  if  possible.  Acute  withdrawal  and  treatment  by  atropin 
for  the  vagotonic  collapse,  may  be  practised  to  advantage. 

Cocain. — Cocain  is  taken  for  the  same  reasons  that  alcohol,  mor- 
phin and  other  drugs  are  taken,  but  it  is  much  more  dominating 
in  its  influence  and  more  difficult  to  escape  from  than  either  of  the 
others,  while  it  is  more  disintegrating  than  morphin  or  opium. 

Symptoms.  —  The  symptoms  of  cocain  intoxication  are  those  of 
marked  stimulation.  The  patient  is  extremely  active  and  very  talka- 
tive, full  of  all  sorts  of  schemes,  ambitious,  tells  what  he  is  going  to 
do,  unfolds  plans,  and  in  general  is  in  a  manic  condition  of  mind. 
Following  this  hyperstimulation  there  is  of  course  profound  malaise 
and  exhaustion.  Associated  with  the  habitual  use  of  cocain  there  is 
more  apt  to  be  marked  and  persistent  sensory  disturbances  in  the 
form  of  paresthesias,  while  in  the  mental  sphere  the  disintegration 
of  the  individual  is  much  more  marked  than  with  opium,  as  a  rule. 
The  moral  sense  is  blunted,  he  lies  readily,  the  judgment  is  impaired, 
and  not  infrequently  they  develop  distinct  delusions  of  which  the 
delusion  of  marital  infidelity  is  not  infrequent.  Chronic  paranoid 
conditions,  hallucinatory  states,  and  deliria  are  seen  in  cases  of  long- 
continued  habituation.  The  drug  may  be  withdrawn  much  more 
rapidly  than  morphin. 

Miscellaneous  Intoxicants. — A  large  number  of  drugs  are  taken 
habitually,  particularly  the  whole  group  of  pain-relieving  and  sleep- 
producing  drugs.  In  general  the  mental  condition  due  to  these  drugs 
varies  all  the  way  from  delirium  as  the  result  of  an  acute  intoxication 
to  chronic  paranoid  conditions.  The  main  feature  of  the  drug  deliria 
is  their  dream-like  character.  The  patients  have  all  sorts  of  grotesque 
experiences  which  they  weave  into  a  more  or  less  consistent  descrip- 
tion, elaborating  the  details  here  and  there  as  may  be  necessary.  The 
delirious  experiences  pass  like  moving  pictures  before  the  patient's 
mind,  and  they  not  infrequently  are  perfectly  composed  as  they 
recount  them,  no  matter  how  grotesque  or  unusual  or  even  terrifying 
some  of  them  may  be. 

Bromides. — The  possibility  of  bromide  delirium  from  the  taking 
of  large  doses  of  bromides  for  a  long  period  of  time  should  be  borne 


GENERAL  CONSIDERATIONS  729 

in  mind.  Patients  are  not  infrequently  admitted  to  hospitals  sup- 
posedly suffering  from  some  psychosis  but  with  a  bromide  delirium,  the 
bromide  having  been  administered  to  quiet  the  nervousness  of  a 
neurosis,  or  perhaps  given  in  the  treatment  of  an  epilepsy.  The 
average  practitioner  does  not  appear  to  realize  the  possibility  of  chronic 
poisoning  that  prescriptions  of  this  sort  present. 

Carbon  Monoxide. — Carbon  monoxide  delirium  is  of  special  impor- 
tance in  connection  with  the  frequent  attempts  at  suicide  with  illuminat- 
ing gas  that  one  meets  with  in  our  large  cities.  The  principal  thing  to 
be  thought  of  in  connection  with  carbon  monoxide  poisoning  is  that 
after  the  initial  effects  of  the  poisoning  have  subsided  and  the  patient 
comes  out  of  the  coma  there  may  be  quite  a  rapid  return  to  normal 
and  the  patient  be  in  an  apparently  normal  condition  for  a  week  or 
ten  days  and  then  a  relapse  occur  with  marked  mental  disturbance. 
The  patient  should  never  be  discharged  from  the  hospital  until  after 
this  period  of  danger  has  been  passed.  Delirium  is  characteristic  of 
this  form  of  poisoning,  and  an  amnesia  for  the  period  usually  follows, 
often  associated  with  fabrications  and  pseudoreminiscences,  producing 
a  Korsakow  syndrome.  Disturbances  of  speech  are  also  common,  as 
are  disturbances  in  the  emotional  field,  such  as,  for  example,  causeless 
laughter.  Pathologically  there  appear  to  be  thrombotic  occlusions  and 
hemorrhages  which  characteristically  are  found  in  the  basal  ganglia, 
and  in  this  region  may  account  for  the  disturbances  of  mimic. 

Lead. — ^In  chronic  lead  poisoning  we  may  find  a  condition  of  pseudo- 
general  paresis  or  Korsakow's  syndrome  in  addition  to  the  ordinary 
hallucinatory  and  delirious  episodes. 

Mercury. — This  poison  produces  characteristically  a  condition  of 
great  irritability  associated  with  insomnia  and  anxiety.  This  condi- 
tion may  become  more  pronounced  and  develop  into  a  well-marked 
delirium. 

There  are,  of  course,  other  poisons,  but  these  are  the  principal  ones. 

General  Considerations. — With  regard  to  all  of  the  habit-forming 
drugs,  particularly  alcohol,  opium,  cocaine,  and  the  analgesics  and 
hypnotics,  it  may  be  said,  as  already  indicated  in  discussing  them 
separately,  that  the  true  cause  of  addiction  lies  in  the  make-up  of  the 
individual.  This  cause  would  appear  to  be  an  auto-erotic  or  narcis- 
sistic fixation.  This  fixation  and  its  results  are  by  no  means  simple 
matters  psychologically  and  differ  very  materially  in  different  individ- 
uals. The  only  hope  for  the  treatment  of  this  group  of  cases,  however, 
is  an  ability  to  modify  this  character  anomaly.  The  treatment,  of 
course,  therefore,  can  only  be  tentative  and  symptomatic  during  the 
periods  of  acute  disturbances,  and  it  is  in  the  interval,  when  the 
patient  is  free  from  the  psychosis,  that  the  treatment  should  be 
applied.  This  is,  of  course,  the  psychoanalytic  treatment  and  should 
be  addressed  to  discovering  the  underlying  etiological  factors.  Unless 
something  can  be  accomplished  in  this  direction  very  little  is  to  be 
hoped  for. 


730  THE   TOXIC  PSYCHOSES. 

As  regards  the  metallic  poisons  associated  with  dangerous  trades,  of 
course,  the  treatment  here  is  purely  symptomatic,  eliminative,  and 
expectant,  while  the  real  effort  that  is  to  be  of  material  help  should  be 
expended  in  prophylaxis. 

Uremia. — In  acute  uremia  the  convulsion  is  one  of  the  most  typical 
signs,  and  cannot  be  distinguished  in  its  outward  manifestations  from 
the  ordinary  epileptic  attack.  Similarly  with  epilepsy,  too,  there  may 
be  quite  well-defined  delirioid  experiences  or  dream  states  in  which 
there  is  disorientation  and  noticeable  disturbance  of  the  sensorium, 
usually  associated  with  more  or  less  constant  activity.  There  may  be 
an  anxious  affect,  or  the  patient  may,  as  is  quite  usual  in  delirium, 
merely  be  interested  in  hallucinatory  experiences.  Acute  uremic 
symptoms,  in  the  mental  sphere,  of  this  character  may  accompany 
exacerbations  of  the  physical  condition  in  a  patient  suffering  from 
chronic  nephritis. 

In  chronic  uremia  one  finds  not  infrequently  marked  mental  symp- 
toms. The  mental  symptoms  are  usually  of  the  character  of  defect,  a 
general  stupidity  with  a  feeling  of  physical  weakness,  desire  to  sleep, 
with  perhaps  complaints  of  headache,  associated  with  irritability. 
Along  with  the  mental  symptoms  may  go  tremor,  speech  disturbances, 
eye  muscle  palsies,  pupillary  differences,  sluggish  or  failing  light  reac- 
tion, which  point  to  an  organic  disease  of  the  brain.  If  along  with  this 
condition  there  are  Jacksonian  attacks  followed  by  mono-  or  hemi- 
plegia with  perhaps  aphasia  and  disturbances  of  vision,  the  similarity  to 
general  paresis  on  the  one  hand  and  to  brain  tumor  on  the  other  may 
be  very  great.  Sometimes  along  with  these  symptoms  the  mood  is 
distinctly  euphoric,  which  still  further  suggests  the  possibility  of 
paresis.  A  certain  number  of  the  cases  of  uremic  psychoses  show  close 
relationships  to  dementia  precox  showing  negativism,  incoherence, 
catatonia.    Probably  this  group  is  more  serious  in  its  prognosis. 

Diabetes  MeUitus. — The  mental  disorder  which  is  associated  with 
diabetes  is  usually  of  a  mild  depressive  type,  often  with  ideas  of  ruin 
and  sin.  Anxiety  states  have  also  been  described.  Occasionally  the 
picture  gives  somewhat  of  the  impression  of  general  paresis.  With 
the  chronic  depressed  condition  paranoid  ideas  sometimes  develop. 

In  general  the  severity  of  the  mental  symptoms  keeps  pace  with  the 
severity  of  the  physical  disease,  as  indicated  by  the  sugar  eliminated. 
When  the  intoxication  is  very  great  the  patient  may  be  irritable, 
sleepy,  stupid,  complain  of  weakness,  and  headache. 

The  diabetic  state  itself  is  an  etiological  factor  in  the  production  of 
arteriosclerosis,  and  evidences  of  arteriosclerosis  may  be  found  asso- 
ciated with  the  condition.  It  should  be  remembered,  however,  that 
both  the  arteriosclerotic  condition  and  the  senile  state  interfere  with 
the  metabolism  of  the  sugars  and,  therefore,  may  themselves  be 
associated  with  glycosuria. 

Gastro-intestinal  Diseases. — In  general  it  is  well  known  that  with 
diseases  below  the  diaphragm  there  is  associated  on  the  mental  side  a 


G ASTRO-INTESTINAL  DISEASES  731 

depressive  mood.  The  relation  between  diseases  of  the  gastro- 
intestinal tract  and  associated  glands  to  mental  states  is  extremely 
difficult  to  interpret.  Many  of  the  gastro-intestinal  disturbances, 
rather  than  being  causes  of  mental  disease,  are  effects.  This  is  par- 
ticularly seen  in  the  realm  of  the  neuroses  and  the  psychoneuroses, 
and  is  discussed  in  the  chapter  dealing  with  these  conditions. 

Certain  cases  of  acute  confusion  develop  associated  with  profuse 
and  offensive  diarrhea,  a  high  grade  of  indicanuria,  vomiting,  low  fever, 
and  perhaps  mild  albuminuria.  Some  of  these  cases  go  on  to  acute 
delirium,  with  typhoid  state,  profound  exhaustion,  coma,  and  death. 
Just  exactly  where  these  cases  belong  and  what  they  mean  is  not 
altogether  known. 


CHAPTER    XXIIL 
PSYCHOSES  ASSOCIATED  WITH  ORGANIC  DISEASES. 

Apoplexy. — Immediately  following  the  apoplectic  insult  the  patient 
is  quite  commonly  unconscious.  As  the  symptoms  subside  and  the 
patient  begins  to  "come  to"  there  may  be  marked  disorientation  to 
the  extent  of  a  mild  delirium,  especially  if  fever  is  present.  This 
symptom  of  a  slight  rise  in  temperature  with  onset  of  mild  delirium 
sometimes  occurs  a  few  days  after  the  original  insult  and  is  then  of 
bad  prognostic  omen.  In  severe  attacks  the  unconsciousness  may 
pass  on  into  profound  coma  and  death. 

Ordinarily  following  an  apoplectic  insult  after  recovery  from  the 
acute  symptoms  a  condition  of  more  or  less  impairment  is  left,  and 
when  it  is  borne  in  mind  that  the  patients  who  suffer  from  apoplexy 
are  in  the  main  in  the  senile  or  arteriosclerotic  period  it  will  be  under- 
stood that  the  symptoms  of  the  defect  take  on  characteristically  symp- 
toms of  these  two  conditions,  which  perhaps  become  aggravated 
materially  following  the  insult.  The  defect,  however,  may  appear  to 
be  very  much  greater  than  it  really  is,  because  of  the  inability  of  the 
patient  to  express  himself  owing  to  incident  aphasia  or  apraxia.  The 
emotional  attitude  of  the  patients  is  usually  one  of  irritability,  although 
indifference  also  enters  into  the  picture.  Such  patients  often  lie 
quietly  and  apparently  indifferent  until  an  attempt  is  made  to  com- 
municate with  them.  Under  these  circumstances  if  they  have  serious 
aphasic  or  apraxic  disturbance  and  know,  for  example,  what  they  wish 
to  say,  they  may  become  very  much  excited  and  quickly  fatigued  and 
emotional  as  a  result  of  their  repeated  efforts  and  failures  to  enter 
into  communication.  This  is  also  quite  characteristic  in  the  younger 
patients  with  apractic  disturbances  and  relatively  clear  intelligence. 
It  is,  so  to  speak,  "maddening"  not  to  be  able  to  control  any  longer 
the  power  of  expression.  Some  of  these  patients,  especially  those  who 
suffer  from  sensory  forms  of  aphasia,  being  out  of  communication  with 
their  fellows,  and  not  being  able  to  understand  what  goes  on  about 
them,  may  acquire  a  suspicious  attitude  which  gives  a  paranoid  color- 
ing to  their  mental  condition.  It  can  be  understood  that  the  apoplexy 
which  produces  disorders  of  expression  and  interferes  with  the  patient's 
keen  contact  with  his  fellows  will  not  only  precipitate  the  deteriora- 
tion of  the  senium,  but  will  make  that  deterioration  much  more  rapid. 

The  neurological  signs  of  hemiplegia  are  present,  also  often  the 
signs  of  senility,  and  not  infrequently  of  arteriosclerosis,  while  a  certain 
few  of  these  patients  develop  epileptic  attacks.     (See  Chapter  XII.) 


TRAUMATIC  PSYCHOSES  733 

Traumatism. — The  effects  of  trauma  may  be  divided  into  the  im- 
mediate or  acute  effects  and  the  more  remote  or  chronic  results,  into 
which  the  former  may  or  may  not  merge.  The  ultimate  results  are 
divisible  into  changes  in  constitution  and  defect  conditions,  while  a 
certain  proportion  of  cases  develop  psychoses  following  an  injury 
without  there  being  any  special  connection  between  the  two  or  in 
which  the  injury  can  only  be  conceived  as  a  precipitating  or  contribu- 
tory factor.  Oftentimes  the  injury  is  the  result  rather  than  the  cause, 
as  is  so  frequently  seen  in  paresis,  the  injury  serving  to  call  attention 
to  the  presence  of  a  disease  up  to  that  time  not  recognized. 

The  usual  immediate  result  of  a  head  injury,  either  direct  or  indirect, 
is  a  certain  degree  of  unconsciousness  which  may  vary  all  the  way 
from  a  light  stupor  to  profound  coma.  When  the  unconsciousness  is 
not  profound  the  patients  are  quiet,  but  if  disturbed  become  very 
irritable  and  resistive.  Quite  typically,  associated  with  the  stupor, 
is  a  mild  delirium  of  hallucinatory  character  which  is  especially  pro- 
nounced and  apt  to  be  more  violent  in  patients  who  are  the  subject 
of  alcoholism.  Associated  with  this  may  be  a  slight  rise  of  temperature, 
but  if  the  rise  is  pronounced  meningitis  should  be  thought  of.  Alternat- 
ing conditions  of  apparent  clearness  and  marked  confusion  may  occur. 
Usually  the  stupor  is  of  only  short  duration,  but  may  last  several  days 
and  finally  disappear.  In  severe  cases  the  patient  becomes  comatose 
and  dies  in  that  condition.  In  a  few  cases  the  delirious  reaction  is 
unusually  prolonged  and  may  be  associated  with  Korsakow's  syn- 
drome. 

After  recovery  from  the  immediate  effects  of  the  injury  certain 
constitutional  changes  may  slowly  develop,  the  most  typical  of  which 
is  Friedmann's  complex,  which  he  attributes  to  a  vasomotor  disturb- 
ance in  the  brain.  The  symptoms  of  this  complex  are  headache,  dizzi- 
ness, irritability,  insomnia,  physical  and  mental  fatigability,  a  certain 
change  of  character,  and  intolerance  to  alcohol,  with  which  may  be 
associated  a  memory  defect  of  the  type  of  retrograde  amnesia,  which, 
however,  is  not  a  part  of  the  complex,  although  there  may  be  a  certain 
memory  defect  which  is  quite  common  to  all  highly  nervous  worried 
people.  The  headache  is  apt  to  be  complained  of  as  a  feeling  of  pain 
or  a  peculiar  feeling  of  constriction  of  the  head  and  is  associated  with 
feelings  of  fulness,  especially  on  stooping  over.  The  dizziness  is  also 
characteristically  intensified  by  stooping  or  by  sudden  movements. 
The  irritability  of  temper  may  be  so  marked  as  to  lead  to  explosive 
outbreaks,  which  simulate  psychic  epilepsy.  Kaplan  has  named  this 
condition  the  explosive  diathesis.  They  are  much  aggravated  by 
alcohol.  Sleep  is  liable  to  be  disturbed,  phj^sical  and  mental  endurance 
is  very  much  lessened,  and  the  patient  may  become  depressed,  moody, 
and  in  various  other  ways  show  a  subtle  change  in  character  which  is 
appreciated  by  all  of  his  close  associates.  The  intolerance  to  alcohol 
is  very  characteristic  of  this  post-traumatic  constitution.  Sometimes 
associated  with  the  explosive  diathesis,  but  also  occurring  alone,  are 


734       PSYCHOSES  ASSOCIATED  WITH  ORGANIC  DISEASES 

temporary  dream  states.  Various  other  hysteriform  and  epileptiform 
manifestations  may  also  occur. 

Finally,  certain  defects  are  left  as  a  result  of  the  traumatism,  depend- 
ing, of  course,  upon  the  nature,  the  extent,  and  the  location  of  the 
injury.  As  regards  the  more  characteristic  results  of  head  injury  of  a 
distinctly  psychotic  nature,  it  should  be  borne  in  mind  that  the  change 
in  character,  transformation  of  the  personality,  as  it  might  be  called, 
is  often  a  very  subtle  process  and  one  extending  over  a  very  consider- 
able period  of  time.  It  might  be  practically  impossible  to  evaluate 
the  situation  at  all  if  one  were  dependent  upon  a  cross-section  of  the 
patient's  mental  life.  When  a  longitudinal  section,  however,  is  avail- 
able one  finds  in  typical  cases  an  individual  who  up  to  a  certain  point 
in  life  has  gotten  along  well,  showing  efficient  reactions  and  developing 
by  steady  progress  in  some  chosen  line  of  work.  Such  an  individual 
receives  a  head  injury  and  from  the  time  of  this  injury  on  there  will 
be  noted  in  the  history  a  gradual  falling  off  in  efficiency.  It  may  be 
quite  impossible  to  put  one's  finger  upon  any  specific  thing  in  the 
situation  and  set  it  down  as  a  pathological  type  of  reaction,  but  the 
individual  reaction  has  changed  in  character,  and  from  efficiency  there 
is  evolved  inefficiency.  Naturally,  it  is  a  long  time  before  the  true 
explanation  of  such  a  change  is  reached.  In  fact  it  is  a  long  time  before 
it  is  realized  that  any  change  at  all  has  taken  place,  and  it  is  because 
of  this  fact  that  the  older  psychiatrists  used  to  speak  of  traumatic 
insanity  as  eventuating  many  years  sometimes  after  the  injury.  Not 
infrequently  this  falling  off  in  efficiency  has  going  with  it  a  gradual 
deterioration  in  morale,  and  with  the  heightened  susceptibility  of  the 
patient  to  alcohol,  which  trauma  produces,  it  is  natural  that  alcohol 
should  enter  into  the  picture  very  largely  and  often  be  regarded  as  the 
cause  of  the  whole  trouble. 

Meyer's^  classification  of  the  effects  of  traumatism  in  the  nervous 
system  is  as  follows : 

1.  The  direct  focal  and  the  more  diffuse  destruction  of  the  nerve 
tissue  or  of  parts  of  it;   and  the  reaction  of  the  tissues. 

(a)  The  immediate  effects — edema. 

(b)  The  scar  formation. 

2.  The  distinctly  diffuse  commotions  in  which  the  general  reaction 
and  the  psychic  elements  preponderate,  including  the  remote  reactive 
results  of  exaggerations  of  vasomotor  and  emotional  responsiveness. 

He  classifies  the  psychoses  developing  as  follows: 

1.  •  The  direct  post-traumatic  deliria  with  the  following  subdivisions: 

(a)  Preeminently  febrile  reactions. 

(b)  The  delirium  nervosum  of  Dupuytren,  not  differing  from  deliria 
after  operations,  injuries,  etc. 

(c)  The  delirium  of  slow  solution  of  coma  with  or  without  alcoholic 
basis. 

1  The  Anatomical  Facts  and  Clinical  Varieties  of  Traumatic  Insanity,  Amer.  Jour. 
Insanity,  January,  1904. 


CHOREIC  PSYCHOSES  735 

(d)  Forms  of  protracted  deliria  usually  with  numerous  tabulations, 
etc.  (with  or  without  alcoholic  or  senile  basis). 

2.  The  post-traumaiic  constitution. 

(a)  Types  with  mere  facilitation  of  reaction  to  alcohol,  grippe,  etc. 

(b)  Types  with  vasomotor  neurosis. 

(c)  Types  with  explosive  diathesis. 

(d)  Types  with  hysteroid  or  epileptoid  episodes  with  or  without 
convulsions  (such  as  most  reflex  psychoses). 

(e)  Types  of  paranoiac  development. 

3.  The  traumatic  defect  conditions. 

(a)  Primary  defects  allied  to  aphasia. 

(b)  Secondary  deterioration  in  connection  with  epilepsy. 

(c)  Terminal  deterioration  due  to  progressive  alterations  of  the 
primarily  injured  parts,  with  or  without  arteriosclerosis. 

4.  Psychoses  in  which  trauma  is  merely  a  contributory  factor. 

(a)  General  paralysis  with  or  without  traumatic  stigmata. 

(b)  Manic-depressive  and  other  transitory  psychoses,  catatonic 
deterioration  and  paranoiac  conditions,  with  or  without  traumatic 
stigmata. 

5.  Traumatic  psychoses  from  injury  not  directly  affecting  the  head. 
This  group  of  traumatic  psychoses  includes  also  a  certain  number  of 

the  postoperative  psychoses,  psychoses  following  operations  upon  the 
eye  and  residence  in  a  dark  room,  the  so-called  ophthalmic  psychosis 
and  the  psychoses  of  insolation.  Of  course  this  statement  applies  only 
to  such  psychoses  as  do  not  belong  in  other  groups,  as  the  shock  of 
surgical  operations,  etc.,  may  well  be  a  precipitating  factor  in  the  onset 
of  such  psychoses.  When,  however,  they  are  essentially  psychoses 
the  result  of  shock  they  have  a  symptomatology  and  history  quite 
the  same  as  the  traumatic  psychoses.  They  can  be  understood  if  the 
hypothesis  of  Friedmann  is  borne  in  mind  that  the  complex  named  after 
him  is  due  to  vasomotor  disturbance.  If  this  be  true  it  can  be  under- 
stood how  severe  emotional  traumas,  as  well  as  physical  traumas  may 
produce  a  vasomotor  imbalance,  although,  of  course,  it  must  never  be 
forgotten  that  whatever  may  in  the  last  analysis  be  the  actual  cause, 
the  cause  operates  upon  a  certain  kind  of  individual,  and  the  symptom 
picture  is  necessarily  modified  accordingly. 

Acute  Chorea  (Sydenham's). — Quite  usually  a  patient  with  acute 
chorea  is  irritable,  somewhat  emotionally  unstable,  fretful,  and  im- 
patient, a  state  of  mind  one  would  expect  from  the  nature  of  the 
illness.  While  this  condition  is  quite  usual  it  may  become  somewhat 
more  aggravated,  the  restlessness  and  emotional  instability  be  more 
pronounced,  with  evidences  of  transitory  disturbances  in  the  sensorium, 
and  perhaps  slight  apprehensiveness  and  suspiciousness.  The  hallu- 
cinatory disturbances  may  become  very  much  aggravated,  together 
with  clouding  of  consciousness  and  marked  delirium  in  connection 
with  the  febrile  movement.  Under  these  circumstances,  of  course, 
we  are  dealing  essentially  with  a  fever  delirium.     Along  with  these 


736       PSYCHOSES  ASSOCIATED  WITH  ORGANIC  DISEASES 

milder  manifestations  of  mental  disorder  one  occasionally  finds  symp- 
toms of  a  hysteriform  character. 

Chorea  insaniens  is  generally  considered  as  a  distinct  form  of  acute 
chorea.  It  is  associated  with  high  temperature  and  marked  mental 
disturbances,  usually  beginning  early  in  the  disease.  The  mental 
disorder  is  essentially  of  a  delirious  character,  with  hallucinations  and 
clouding  of  consciousness.  The  hallucinations  are  quite  apt  to  be 
terrifying,  and  the  patient  consequently  apprehensive  and  fearful, 
although  the  opposite  condition  of  elation  has  been  described.  The 
disease  is  quite  frequently  associated  with  pregnancy,  and  is  not 
infrequently  fatal. 

Korsakow's  psychosis  is  sometimes  seen  in  patients  suffering  from 
acute  chorea  as  a  result  of  polyneuritis  resulting  from  overtreatment 
with  arsenic.     (See  Section  on  Chorea.) 

Chronic  Chorea  (Huntington's).  —  It  has  always  been  recognized 
that  this  disease  was  associated  with  mental  symptoms,  and  it  has 
generally  been  considered  that  there  was  a  tendency  to  progressive 
deterioration  ending  in  well-marked  dementia.  While  in  a  general 
way  this  may  be  true,  it  is  well  to  be  cautious  in  estimating  the  mental 
condition  of  chronic  choreics.  They  are  extremely  inaccessible  in  many 
instances,  especially  those  patients  whose  speech  apparatus  is  affected 
by  the  disorder,  and  it  requires  great  patience  to  make  a  satisfactory 
examination  of  their  mentality.  On  the  other  hand  the  patients 
themselves  find  extensive  explanations  so  difficult  to  communicate 
that  they  are  content  with  the  shortest  possible  responses,  so  that 
they  frequently  mislead  the  examiner  into  the  belief  in  an  intellectual 
poverty  which  does  not  really  exist. 

With  these  warnings  it  may  be  conceded  that  in  general  the  chronic 
choreic  is  of  somewhat  enfeebled  mentality  with  a  tendency  to  emo- 
tional depression  and  to  a  mood  of  suspiciousness.  The  emotional 
depression  can  be  readily  understood,  as  the  patient  realizes  quite 
well  his  plight,  namely,  that  he  is  affected  with  a  chronic  disease 
which  practically  isolates  him  from  his  fellows.  Not  infrequently 
there  is  a  marked  emotional  instability  and  irritability,  and  such 
choreic  patients  may  have  great  difficulty  in  getting  along  in  wards 
where  they  come  in  contact  with  other  patients.  Finally,  with  the 
increasing  deterioration  there  is  a  tendency  to  indifference,  to  emo- 
tional deterioration.  The  suspicious  mood,  which  so  many  of  these 
patients  have,  we  believe  has  at  least  in  part  its  explanation  in  the 
patient's  isolation  as  a  result  of  the  nature  of  the  disease.  We  find 
suspicious  moods  in  all  persons  who  are  cut  off  from  active  contact 
with  their  fellows,  more  especially  in  people  who  are  closely  confined, 
either  voluntarily  or  otherwise,  those  who  suffer  from  pronounced 
varieties  of  aphasia,  which  make  it  impossible  for  them  to  communicate 
their  thoughts  or  to  receive  communications  from  others,  and  pre- 
dominantly among  the  deaf.  To  a  certain  extent,  too,  the  dementia 
is  probably  due  to  this  cutting  off  of  active  contact  with  life  by  im- 


MULTIPLE  SCLEROSIS  AND  PSYCHOSES  737 

pairing  the  means  of  communication.  The  choreic  finds  it  extremely 
difficult  to  talk  to  others.  He  may  find  it  equally  difficult  to  read, 
perhaps  because  of  defect  of  vision  which  is  liable  to  be  present,  as 
it  must  be  remembered  that  this  is  a  disease  of  later  life,  and  in  various 
ways  he  loses  touch  with  what  is  going  on  about  him.  Finally,  when 
he  has  long  passed  any  ability  to  pick  up  the  thread  of  events  he 
becomes  indifferent,  and  when  it  is  realized  that  he  is  frequently  in 
the  arteriosclerotic  period,  often  approaching  the  senium,  it  can  be 
seen  that  this  removal  from  acute  contact  with  reality  tends  to  produce 
inactivity,  as  it  were,  and  appearances  of  dementia,  if  not  actual 
dementia,  and  so  the  chronic  choreic  shows  emotional  defects,  attention 
disorder,  lack  of  impressibility,  defect  of  recall,  and  in  other  ways 
demonstrates  that  he  has  lost  interest,  as  it  were,  in  life. 

A  certain  few  cases  show  distinctly  more  pronounced  psychotic 
symptoms,  occasionally  showing  well-marked  persecutory  ideas 
(See  Chapter  X.) 

Paralysis  Agitans. — It  is  probable  that  the  majority  of  cases  of 
paralysis  agitans  show  a  certain  amount  of  mental  disturbance, 
although  in  a  great  number  of  cases  this  disturbance  is  so  slight 
compared  with  the  physical  and  so  easily  seen  to  be  dependent  upon 
it  that  it  is  quite  overlooked.  This  simple  disturbance  is  in  the 
emotional  sphere  and  for  the  most  part  is  one  of  slight  depression 
but  rarely  one  of  euphoria.  Occasionally  deliriod  episodes  supervene, 
with  marked  confusion,  but  in  general  consciousness  is  clear  and  the 
patient  remains  well  oriented,  except  of  course,  in  the  terminal  con- 
dition when  arteriosclerotic  changes  and  the  deterioration  of  the 
senium  have  set  in.  Occasionally  there  is  a  well-marked  psychosis 
in  which  instance  it  is  most  apt  to  be  of  a  depressive  hypochondriacal 
character,  often  with  paranoid  coloring.  The  depression  may  be 
sufficiently  great  to  result  in  efforts  at  suicide.  In  these  cases  disorders 
of  the  sensorium  may  also  be  present. 

As  intimated  above  it  must  be  remembered  that  we  are  dealing 
with  a  disease  of  later  life  and  that  not  infrequently  arteriosclerotic 
and  senile  changes  show  themselves  by  changes  in  the  mental 
sphere. 

Multiple  Sclerosis. — The  outward  manifestations  of  the  mental 
disorder  associated  with  this  disease  are  mainly  in  the  emotional 
sphere.  It  has  been  stated  both  that  the  majority  of  patients  were 
depressed  and  that  the  majority  were  exalted.  Both  these  statements 
are  based  upon  the  observation  of  very  few  patients.  Although  exalta- 
tion and  depression  may  be  manifest  and  there  may  also  be  spasmodic 
laughing  and  crying,  due  probably  to  lesions  of  the  basal  ganglia, 
which  are  unaccompanied  by  emotion,  there  is  a  certain  amount  of 
deterioration  which  is  progressive  and  which  depends  of  course  upon 
the  destructive  changes.  When  the  distribution  of  the  sclerotic 
patches  is  quite  similar  to  the  distribution  of  the  lesions  in  general 
paresis  the  outward  symptoms  of  the  two  conditions  may  be  very 
47 


738       PSYCHOSES  ASSOCIATED   WITH  ORGANIC  DISEASES 

much  alike  and  lead  to  confusion  in  diagnosis.  The  diagnostic 
problems,  however,  can  be  cleared  up  by  the  laboratory  findings. 

Polyneuritis. — See  Korsakow's  Psychosis. 

Pellagra. — There  has  been  very  little  study  of  the  mental  symptoms 
of  pellagra  of  late  years.  From  the  few  cases  observed  and  from 
study  with  those  who  have  had  it  under  observation  there  seems  to 
be  a  variety  of  symptom  pictures. 

Many  cases  present  no  nervous  or  mental  symptoms  at  all.  In 
those  who  do  there  seems  to  be  a  tendency  toward  a  variable  localiza- 
tion of  the  disease  process.  There  seems  to  be  cases  in  which  the 
spinal  cord  suffers  most  and  others  in  which  the  brain  suffers  most.  In 
this  latter  group  a  condition  of  very  acute  delirium  may  be  developed 
running  a  rapid  course  to  fatal  termination  and  reminding  one  of  the 
acute  forms  of  paresis.  The  more  frequent  condition,  of  which  we 
have  seen  a  number  of  cases,  seems  to  be  more  in  the  nature  of  a 
simple  retardation.  The  patient  moves  slowly,  or  not  at  all,  and 
answers  questions  after  a  long  delay  in  a  low  tone  of  voice  and  in 
monosyllables.  There  does  not  go  with  this  retardation,  however, 
a  corresponding  emotional  depression  as  in  melancholia.  We  have 
also  seen  pellagrophobia  in  an  infected  territory. 

With  this  disease,  as  with  many  others,  it  must  not  be  forgetten  that 
it  may  be  associated  with  various  psjxhoses  without  having  any  specific 
relation  to  them.  This  is  peculiarly  so  in  this  country,  as  the  large 
groups  of  cases  which  have  occurred  have  been  in  hospitals  for  the 
insane. 

Perhaps  Gregor^  has  made  the  most  careful  recent  clinical  study 
of  the  mental  symptoms.  He  considers  his  cases  under  the  following 
seven  categories:  (1)  Pellagrous  neurasthenia,  (2)  stuporous  group, 
(3)  mental  aberration,  (4)  acute  delirium,  (5)  katatonia,  (6)  anxiety 
psychosis,  (7)  manic-depressive  group.  It  does  not  seem  clear,  how- 
ever, just  what  is  the  connection  in  all  of  these  cases  between  the 
pellagra  and  the  psychosis. 

Heart  Disease. — Depressive  states  are  most  in  evidence  here. 
Mental  symptoms,  however,  are  most  apt  to  be  associated  with 
failure  of  compensation.  With  precordial  distress  goes  typically 
a  mental  state  of  anxiousness.  Transitory  confusions  with  dreamy 
hallucinations  occur  with  compensation  disturbances  and  edema. 

Various  other  diseases  have  from  time  to  time  mental  symptoms 
associated  with  them.  The  great  majority  of  such  diseases,  if  not  all 
of  them,  have  elements  of  infection,  or  toxemia  and  exhaustion  com- 
bined, with  all  or  part  of  them.  The  mental  symptom-complex  of 
confusion  arises  most  typically.  In  some  cases,  especially  the  less 
acute,  paranoid  conditions  occur  and  hallucinosis  is  of  occasional 
occurrence. 

Head  has  shown  that  certain  visceral  diseases,  especially  of  cardio- 

1  Beitrage  zur  Kentniss  der  pellagrosen  Geistesstorungen,  Jahrb.  f.  Psych,  u.  Neurol,, 
1907. 


HEART  DISEASE  AND  PSYCHOSES  739 

vascular  and  pulmonary  origin,  often  have  associated  mental  symp- 
toms, although  they  may  not  appear  except  on  the  most  careful 
examination.  The  symptoms  found  are :  (1)  Hallucinations  of  vision, 
hearing  and  smell;  (2)  moods,  either  of  depression  or  exaltation,  and 
(3)  suspicions  usually  occurring  when  a  depression  has  persisted  for 
some  time. 

These  conditions  take  their  origin  in  part  as  a  result  of  reflected 
visceral  pains.  Each  spinal  segment  has  both  a  visceral  and  a  cutane- 
ous representation.  Disease  occurring  in  the  visceral  area  is  referred 
to  the  cutaneous  surface  supplied  by  the  same  segment.  The  cutaneous 
distribution  of  the  fifth  nerve  corresponds  to  the  visceral  distribution  of 
the  vagus,  so  pain  occurring  in  the  vagus  territory  will  be  referred  to  the 
scalp  and  thus  occur  points  of  tenderness  in  this  region  with  which  the 
hallucinations  are  associated.  The  mood  of  exaltation  is  essentially 
transitory  and  arises  as  a  contrast  phenomenon  of  the  depression  and 
as  a  result  of  the  disappearance  or  lessening  of  the  reflected  somatic 
pain. 


CHAPTER   XXIV. 

PRESENILE,   SENILE,  AND  ARTERIOSCLEROTIC 
PSYCHOSES. 

The  grouping  of  the  presenile,  senile,  and  arteriosclerotic  psychoses 
together  in  one  chapter  is  a  matter  not  only  of  convenience,  for 
obviously  one  would  expect  all  sorts  of  admixtures,  particularly  as 
between  the  senile  and  arteriosclerotic  group,  but  there  are  many 
pictures  here  which  are  not  distinctive,  especially  in  the  presenile 
period,  which  Kraepelin  begins  his  discussion  of  by  saying  it  is  the 
darkest  region  of  all  psychiatry  today. 

The  Presenile  Psychoses. — In  the  presenile  period  there  are  a 
number  of  psychotic  pictures  the  exact  significance  and  nosological 
placement  of  which  is  not  at  all  understood.  There  is  no  doubt,  how- 
ever, but  in  this  period  that  depressions  are  much  more  in  evidence 
than  excitements,  the  depressions  seeming  to  mount  up  in  frequency 
during  the  period  of  involution.  Hiibner  found,  for  example,  21  cases 
of  single  attacks  of  melancholia  after  the  fiftieth  year,  but  only  2  cases 
of  single  attacks  of  excitement. 

Involution  Melancholia. — Symptoms. — The  Kraepelin  school  for  a 
considerable  time  considered  that  certain  depressions  of  later  life, 
characterized  by  an  anxious  apprehensive  agitation,  with  pro- 
found emotional  depression,  composed  a  nosological  unit  to  which 
was  given  the  name  involution  melancholia,  or  more  briefly  melan- 
cholia, the  term  melancholia  being  limited  in  its  application  to  this 
particular  type  of  depression. 

The  symptoms  of  this  psychosis  are  generally  preceded  by  a  con- 
siderable period  during  which  there  are  vague  head  symptoms,  such 
as  pressure,  pain,  vertigo,  together  with  anorexia,  irritability,  insomnia, 
mental  insufficiency,  and  a  mild  neurastheniform  state  with  perhaps 
some  emaciation.  This  condition  becomes  progressively  worse  and 
the  patients  develop  an  agitated  depression,  with  anxiety,  apprehen- 
sion, fear  of  impending  danger,  with  quite  usually  delusions  of  sin. 
The  depression  is  characteristically  very  profound,  and  the  motor 
agitation  may  be  quite  considerable.  The  patient  may  go  about 
wringing  his  hands,  moaning  and  groaning,  perhaps  repeating  over 
and  over  again  such  phrases  as  "Oh,  my  God!  Oh,  my  God!"  "It 
is  a  fearful  thing.  Good  Lord  help  me!"  One  patient  constantly 
repeats  "Doctor,  will  I  be  done  away  with  tonight?"  and  "Then  will 
I  be  here  tonight  just  the  same  as  last  night,  and  will  I  be  here  tomorrow 
just  the  same  as  today?"    Another  patient  believes  herself  very  sin- 


THE  PRESENILE  PSYCHOSES 


741 


ful,  refuses  to  eat  because  the  food  should  be  used  for  others,  walks 
about  in  an  agitated  manner  picking  her  fingers  and  attempted  suicide 
because  she  was  afraid  she  was  to  be  put  to  death. 

Even  in  these  cases  of  quite  extreme  agitation  and  profound  depres- 
sion consciousness  remains  unclouded,  orientation  is  little  if  at  all 
impaired,  and  the  form  of  thought  is  well  maintained. 

Another  type  of  case  presents  symptoms  of  retardation.  Many 
of  these  cases  may  be  so  retarded  as  to  be  almost  stuporous  and  some 
of  them  present  an  amount  of  resistance  which  reminds  one  of  the 
characteristic  negativism  of  dementia  precox.  This  latter  group  was 
originally  described  by  Kraepelin  as  "late  catatonia."  Its  nosological 
status  is  at  present  in  doubt. 

Certain  cases  of  anxious  depression  may  be  highly  agitated  with 
insomnia,  refusal  of  food,  rapid  emaciation,  clouding  of  consciousness, 
hallucinations,  self-inflicted  injuries,  attempts  at  suicide,  a  rapid 
course  ending  in  death. 


Fig.  313. — Facies  of  involution  melancholia. 


In  the  marked  cases  of  apprehensive  and  agitated  depression  there 
is  frequently  a  considerable  degree  of  precordial  distress  with  perhaps 
tachycardia  and  often  a  sense  of  oppression  over  the  chest  with  a 
feeling  of  difficulty  of  breathing.  These  symptoms  are  apt  to  appear 
in  attacks,  at  which  times  the  depression  is  more  pronounced. 

The  danger  from  suicide  is  very  great  in  this  psychosis,  particularly 
because  the  patient  is  not  so  frequently  rendered  inactive  by  marked 
retardation. 

The  delusional  content  of  consciousness  varies  widely,  but  hypo- 
chondriacal delusions,  nihilistic  delusions,  and  the  feeling  of  unreality 
are  common.  Occasionally  the  delusions  take  on  bizarre,  absurd  and 
fantastic  forms,  and  there  may  be  delusions  of  grandeur  late  in  the 
course  of  the  disease.  There  may  be  a  strange  mixture  of  depressive 
and  grandiose  ideas,  as  in  the  patient  of  Weygandt,  who  believed  she 
was  going  to  be  boiled  in  a  silver  kettle. 

The  statistics  of  this  disease  show  that  about  40  per  cent,  got  well, 
the  remaining  60  per  cent,  terminating  in  various  ways:  some  by 


742     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

suicide,  some  by  death  from  intercurrent  disease,  which  they  were 
ill  adapted  to  withstand;  some  by  death  from  general  marasmus  or 
the  development  of  tuberculosis;  some  lapsed  into  chronicity;  and 
finally,  a  few  improved  sufficiently  to  leave  the  hospital  and  get  along 
at  home,  though  still  somewhat  depressed.  A  certain  number  of  this 
class  may  get  worse  under  home  surroundings  and  have  to  be  returned 
to  the  hospital. 

This  group  of  involution  melancholia,  so-called,  was  studied  with 
great  care  by  Dreyfus.^  He  studied  the  life  histories  of  81  cases,  34 
of  which  were  personally  investigated.  Eight  were  not  personally 
investigated,  and  39  were  deceased.  As  a  result  of  his  study  he  con- 
cluded that  with  the  exception  of  two  cases  undiagnosed,  two  cases 
in  which  a  mistake  in  diagnosis  had  been  made,  and  possibly  two 
more  doubtful  cases,  that  all  were  cases  of  manic-depressive  psychosis. 
This  conclusion  was  reached  by  finding  the  fundamental  symptoms 
of  this  disease  present.  Of  these  cases  66  per  cent,  were  recovered  or 
were  recovering  at  the  time  of  death,  8  per  cent,  developed  arterio- 
sclerosis, 25  per  cent,  died  unrecovered  of  recurrent  disease  or  suicide. 

The  conclusion  reached  by  Dreyfus  that  the  involution  melancholia 
of  Kraepelin  is  really  a  form  of  manic-depressive  psychosis  has  now 
been  accepted  by  Kraepelin  himself.  A  study  of  this  group  of  cases 
shows  quite  characteristically  the  presence  in  the  history  of  periods 
of  affect  fluctuation;  and  quite  usually  periods  of  slight  depression 
which  were  not  severe  enough  to  attract  attention  seriously  to  the 
mental  condition,  or  perhaps  were  not  at  all  understood  at  the  time, 
being  accounted  for  in  various  other  ways. 

Even  admitting  that  this  group  of  involution  melancholias  really 
belong  to  the  manic-depressive  psychosis,  still  the  problem  is  not 
wholly  solved  and  it  must  be  admitted  that  in  all  probability  the 
involution  period  has  certain  modifying  effects  upon  the  psychosis. 

In  the  first  place  the  depressions  as  noted  heretofore  are  very  much 
more  frequent  than  the  excitements  and  they  are  very  much  longer  in 
duration  than  during  earlier  life.  This  of  course  can  be  easily  under- 
stood by  the  failing  resistance  of  the  involution  period.  A  psychosis 
that  has  manifested  itself  only  by  mild  and  hardly  appreciable  evidences 
throughout  the  youth  of  the  individual  may  well  get  a  firm  hold  when 
the  powers  of  efficient  reaction  are  failing. 

With  the  recent  work  that  has  been  done  on  the  ductless  glands 
and  with  the  somewhat  characteristic  mental  pictures  that  go  along 
with  disturbances  of  the  internal  secretions  one  must  bear  in  mind 
that  perhaps  many  of  the  changes  of  the  involution  period  are,  in 
part  at  least,  determined  by  changes  in  these  glands,  particularly  as 
the  result  of  atrophy  of  the  uterus,  the  ovaries,  the  prostate,  the 
testicles,  and  the  adrenals,  with  the  possible  result  of  an  imbalance 
being  brought  about  in  the  relationship  between  them.    This  of  course, 

1  Die  Melancholic,  ein  Zustandsbild  des  manisch-depressiven  Irreseins,  Jena,  Gustav 
Fischer,  1907. 


THE  PRESENILE  PSYCHOSES  743 

if  it  is  so,  would  only  be  one  of  the  modifying  factors  of  the  involution 
period  which  one  might  expect  to  see  reflected  in  the  diseases  at  this 
time  of  life. 

The  following  case  illustrates  this  type  of  psychosis:  The  patient, 
a  woman,  was  admitted  to  the  hospital  at  the  age  of  fifty.  She  was 
very  much  depressed,  said  that  she  was  very  unworthy  and  should 
be  hung,  that  she  had  committed  the  unpardonable  sin  and  would 
burn  forever  in  the  flames  of  Hell.  A  little  later,  in  addition  to  these 
ideas  that  she  had  committed  the  unpardonable  sin  and  that  her 
soul  was  lost,  she  said  that  she  was  dead,  what  existed  of  her  now  was 
her  spirit,  that  her  body  had  passed  away,  and  because  she  was  dead 
it  was  useless  to  attempt  suicide.  This  condition  had  its  origin  some 
two  years  before,  following  an  operation,  when  she  had  cleared  up, 
and  as  a  result,  so  stated,  from  overwork,  had  broken  down  again  and 
developed  these  ideas,  and  subsequently  was  admitted  to  the  hos- 
pital after  having  made  a  suicidal  attempt.  In  addition  to  the  delu- 
sions she  had  visual  hallucinations,  saw  spirits  and  heard  them  talking 
to  her. 

This  patient  gave  the  general  impression  on  her  admission  to  the 
hospital  of  a  patient  suffering  from  involution  melancholia.  A  further 
inquiry,  however,  developed  the  history  of  numerous  previous  attacks 
of  depression,  which  suggested  a  manic-depressive  reaction  type. 
Just  before  her  discharge  from  the  hospital,  however,  when  she  had 
become  much  more  accessible,  we  learned  that  in  her  early  life,  before 
she  was  twenty,  she  had  had  a  love  affair.  This  love  affair  had  been 
a  very  profound  emotional  experience,  particularly  because  she  had 
found  it  necessary  to  give  up  the  young  man  because  of  his  drinking 
habits.  That  this  was  not  willingly  done,  however,  was  shown  by  her 
subsequent  life,  which  was  taken  up  by  constant  activities,  largely 
of  a  social  nature,  and  apparently  for  the  purpose  of  side-tracking  her 
disappointment.  She  was  an  attractive  young  woman  and  had  many 
admirers,  but  did  not  permit  herself  to  become  attached  to  any  one 
of  them.  She  led  a  very  active  life  and  was  able  to  sublimate  the 
energy  of  her  suppressed  emotions  until  the  period  of  the  menopause, 
when  she  broke  down  with  self-accusatory  delusions  and  suicidal 
tendencies.  She  believed  she  had  committed  the  unpardonable  sin; 
this  sin  was  having  committed  adultery  in  her  mind  with  the  young 
man  with  whom  she  had  been  in  love  in  her  youth.  The  psychosis 
lasted  somewhat  over  five  years,  at  the  end  of  which  she  finally  suc- 
ceeded in  reaching  a  compromise  by  developing  tendencies  diametri- 
cally opposed  to  her  delusional  system.  She  became  talkative, 
humorous,  and  particularly  facetious,  and  just  before  her  discharge 
she  made  the  remark  that  she  was  born  dead,  but  made  it  with  a  smile, 
and  with  the  added  comment  that  she  had  gotten  along  pretty  well 
all  her  life  for  a  dead  person.  This  condition  of  relative  equilibrium 
enabled  her  to  go  back  to  her  family  and  in  the  absence  of  disturbing 
factors  she  will  probably  get  along  all  right,  at  least  for  a  considerable 


744     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

time.  Nothing  has  been  heard  of  her  for  some  years.  The  compensa- 
tion, the  balance  struck,  was  a  fairly  efficient  one. 

Such  a  case  as  this  illustrates  a  common  type  of  depression  which 
occurs  in  the  involution  period.  It  is  a  type  which  if  dealt  with 
solel}^  from  the  descriptive  standpoint  would  have  been  considered 
an  involution  melancholia,  while  a  little  more  careful  history  indicates 
that  it  is  a  manic-depressive  reaction  type,  while  a  still  more  profound 
probing  into  the  facts  of  the  life  make  the  whole  thing  quite  under- 
standable in  psychological  terms.  It  is  manifestly  a  psychogenetic 
depression,  but  whether  it  should  be  called  manic-depressive  or  not 
is  a  question.  Now  that  psychoanalj-tic  methods  are  showing  the 
presence  of  endopsj^'chic  conflicts  in  the  manic-depressive  psychosis, 
such  a  case  as  this  shows  the  close  connection  between  a  pure  psy- 
chogenetic psychosis  and  the  manic-depressive  reaction  tj-pe,  and  the 
further  relation  of  these  conditions  to  the  depressions  occurring  during 
the  involution  period. 

Pathology. — An  increased  neuroglia  formation  in  the  deeper  layers 
of  the  cortex  has  been  described,  and  in  this  disease  we  find  most 
often  a  termination  in  the  condition  described  by  ]Meyer  as  central 
neuritis.  This  is  essentially  a  parenchymatous  degeneration  with 
swelling  of  the  cell  body,  a  disappearance  of  the  chromophile  sub- 
stance, and  eccentricity  of  the  nucleus.  In  rapidly  fatal  cases  there 
are  evidences  of  extensive  destructive  changes  in  the  cortex.  There 
are  also  found  a  certain  amount  of  disintegration  products. 

Treatment. — One  of  the  very  important  considerations  in  this 
group  of  depressions,  as  shown  by  the  symptomatology,  is  the  preven- 
tion of  suicide.  It  is  therefore  very  much  more  frequently  necessary 
to  interne  these  patients  so  that  they  can  be  properly  watched. 
Insomnia,  agitation,  failure 'of  nutrition  with  suicidal  tendencies  are 
the  conditions  that  have  to  be  met.  For  the  agitation  and  the  insomnia 
hydrotherapy  is  the  best  means  of  treatment.  As  few  drugs  as  possible 
should  be  used.  For  the  suicidal  tendencies  constant  watchfulness, 
which  should  have  as  its  ideal  an  unobtrusiveness  which  does  not 
suggest  the  suicidal  idea  constantly  to  the  patient's  mind.  Failing 
nutrition  must  be  met  by  tube  feeding  if  necessary. 

An  analysis  of  the  patient's  mental  symptoms  with  a  view  to  under- 
standing them  is  of  course  highly  desirable,  but  this  class  of  cases 
do  not  lend  themselves  easily  to  anahi:ic  technique,  at  least  untU  they 
have  begun  to  quiet  down,  oftentimes  not  until  they  are  convalescent. 

Other  Psychoses  of  this  Period.^ — Various  other  types  of  psychoses 
have  been  described  as  belonging  in  this  period.  Types  of  depressive 
Wahnsinn,  some  of  them  associated  with  anxiety  and  proceeding  to 
deterioration,  while  Ivraepelin  has  described  a  very  small  class  of 
so-called  presenile  delusional  psychoses,  affecting  especially  women, 
in  which  delusions  of  infidelit}^  are  usually  prominent,  together  with 
hallucinations.  The  delusions  are  variable  and  do  not  seem  to  at  all 
logically  control  the  conduct.     Farrar  has  described  three  types  of 


THE  PRESENILE  PSYCHOSES  745 

psychoses  belonging  to  this  period:  melancholia  vera,  anxietas  yroe- 
senilis,  and  depressio  apatJietica. 

In  vieJanchoUa  vera  there  is  in  the  main  an  autopsychosis.  The 
dekisions  are  auto-accusatory,  with  ideas  of  sin  but  with  clear  con- 
sciousness; there  is  no  defect  of  orientation.  The  patient  beheves 
his  soul  is  lost,  that  he  is  to  suffer  eternal  torment  hereafter,  and 
about  these  beliefs  there  is  no  doubt,  but  on  the  contrary  a  marked 
"subjective  certainty."  There  may  be  some  slight  tendenc}^  to  soma- 
topsychic delusions,  insight  is  defective,  and  slight  anxiety  may  be 
present. 

In  anxietas  prcesenilis  there  is,  on  the  contrary,  in  the  main  an 
allopsychosis.  There  is  very  marked  ''subjective  uncertainty''  which 
gives  an  unreal  tinge  to  the  outer  world,  and  out  of  which  grows  the 
fear  of  things  unknown,  culminating  in  the  marked  anxiety  which  is 
characteristic  of  this  form  of  the  psychosis.  Remorse  or  dread  of  the 
future  are  not  elements  in  the  depression;  on  the  contrary,  it  is  the 
great  unknown  and  overwhelming  present  that  seems  about  to  destroy 
them.  These  cases  occur  later  in  life  than  the  former,  show  more 
evidences  of  senile  decay,  such  as  arteriosclerosis,  and  present  such 
symptoms  as  verbigeration,  rhythmical  movements,  suggestibility;  the 
prognosis  is  less  favorable. 

In  deijressio  apathetica  there  is  simply  a  let-down,  a  stopping  on  the 
part  of  one  who  has  been  leading  an  active  life.  Interest  abates,  the 
struggle  is  drawn  away  from  and  we  have  a  picture  of  mild  depression 
with  clear  consciousness  and  no  disturbance  of  orientation.  There  is 
some  "subjective  uncertainty"  delusions  and  sensory  fabrications 
play  little  part.  The  symptoms  are  negative  rather  than  positive; 
the  prognosis  is  relatively  good. 

Finally  one  must  remember  that  a  true  anxiety  neurosis  may  develop, 
and  it  not  infrequently  does,  at  this  period  of  life. 

In  conclusion  then  it  will  be  seen  that  we  have  during  this  period 
of  life  a  group  of  psychoses  of  widely  different  forms,  although  tending 
toward  a  certain  amount  of  uniformity  in  their  outward  expression 
in  that  depressions  are  so  much  more  frequent  at  this  period.  In  the 
first  place  there  are  frank  attacks  of  manic-depressive  psychosis  with 
here  as  elsewhere  marked  differences,  depending  upon  the  severity 
of  the  constitutional  taint  upon  the  one  hand,  or  its  absence  and  the 
presence  of  a  reactive  type  upon  the  other.  Apprehension  and 
anxiety  appear  frequently  at  this  period  of  life,  and  a  true  anxietj^ 
psychosis  is  not  uncommon.  Paranoid  conditions  occasionally  occur 
and  disorders  of  the  sensorium  are  not  infrequent.  In  a  certain 
group  of  cases  negativistic  tendencies  are  sufficiently  marked  to  make 
the  outward  semblance  to  catatonia  quite  pronounced. 

Certain  of  the  psychoses  of  this  period  tend  toward  more  or  less 
rapid  deterioration,  and  in  these  at  least  there  are  evidences  of  marked 
physical  changes.  In  other  words,  the  physical  side  of  the  disease 
has  to  be  kept  constantly  in  mind  and  must  be  considered  to  be  the 


746     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

most  important  prognostic  feature.  The  changes  incident  to  vascular 
degeneration,  while  of  course  not  usually  prominent  in  the  fifth  and 
sixth  decades,  may  well  be  in  evidence,  while  the  changes  in  the  duct- 
less glands  are  to  be  borne  in  mind.  Other  changes  undoubtedly  occur, 
but  their  nature  and  their  bearing  upon  the  psychosis  are  not  at  all 
understood.  The  psychosis  must,  nevertheless,  be  expressed  in 
psychological  terms  and  the  explanation  of  the  mental  symptoms  must 
be  sought  by  psychoanalytic  study.  We  have  here,  then,  diseases 
which  have  a  marked  physical  side  apart  from  the  mental  manifesta- 
tions, the  physical  side  being  more  prominent  than  in  dementia  precox 
and  less  prominent  than  in  general  paresis. 

The  Senile  Psychoses. — The  normal  course  of  life  leads  to  a  certain 
amount  of  gradual  mental  and  physical  deterioration  during  the 
latter  years.  Whether  this  occurs  or  not  probably  depends  upon  many 
factors,  for  we  see  some  men  at  sixty  as  old  as  they  should  be  at 
seventy-five,  and  other  people  at  eighty  presenting  a  wonderful  degree 
of  elasticity  and  enthusiasm  without  any  apparent  falling  oft'  in 
mental  powers  or  interests.  This  variation  was  wont  to  be  expressed 
by  saying  that  "a  man  is  as  old  as  his  arteries,"  but  it  is  certain  that 
there  are  many  other  factors  besides  the  condition  of  the  bloodvessels 
that  lead  to  senile  deterioration.  In  fact  senile  deterioration  may 
take  place  and  lead  to  very  profound  dementia  without  material 
disease  of  the  bloodvessels  at  all. 

Intermediate  Conditions. — ^A  certain  number  of  the  involution  psy- 
choses continue  over  into  the  senile  period  when  the  patients  undergo 
the  mental  and  physical  changes  of  senile  involution.  This  is  in  part, 
at  least,  the  reason  for  the  termination  in  dementia  of  a  certain 
group  of  involution  cases,  aside  from  the  added  obvious  fact  that 
vascular  degeneration  is  also  an  important  etiological  moment.  The 
cases  that  show  this  outcome  in  dementia  are  more  especially  the 
paranoid  types.  This  group  of  cases  shows,  therefore,  that  there  is 
a  gradual  transition  from  the  psychoses  of  the  distinctly  involution 
period  to  those  of  the  senile  period,  a  perfectly  understandable  con- 
dition if  we  conceive  of  the  psychotic  manifestations  as  being  expres- 
sions of  mental  conflicts  that  are  at  the  basis  of  the  individual  character 
and  which  express  themselves  in  the  later  years  of  life  when  efficient 
reaction  is  becoming  progressively  less  possible,  and  which  later  on 
become  fixed,  chronic,  and  disintegrating  at  a  time  when  the  physical 
changes  of  the  senium  cooperate  to  this  end. 

Normal  Senile  Involution. — The  more  usual  symptoms  of  senile 
involution  occurring  after  sixty  are  in  the  main  a  loss  of  memory  for 
recent  events,  due  to  lack  of  impressibility  to  the  extent  even  that 
events  of  only  an  hour  before  are  completely  forgotten^  lack  of  ability 
to  recognize  faces,  marked  egotism,  so  that  others'  wants  and  comforts 
are  not  considered,  which  may  be  associated  with  some  irritability 
on  interference.  There  is  developed  more  and  more  as  the  years  go 
on  a  true  misoneism,  so  that  the  patient  will  positively  not  tolerate 


SENILE  INVOLUTION  747 

any  change  in  the  usual  order  of  things,  everything  must  be  done  the 
same  from  day  to  day,  the  same  seat  is  preempted,  a  particular  kind 
of  food  demanded,  and  the  like  with  other  comforts.  With  this  mis- 
oneism  and  the  lack  of  memory  for  recent  events  goes  a  marked 
tendency  to  reminiscence.  The  events  of  youth  and  the  years  long 
past,  unlike  those  of  recent  occurrence,  are  vividly  recalled  and  the 
patient  thus  really  lives  in  a  world  of  former  days,  constantly  recalling 
and  reiterating  things  that  occurred  long  ago.  This  condition  becomes 
progressively  worse,  the  patient  leading  a  vegetative  existence  almost 
wholly,  no  mental  initiative,  failure  of  judgment  and  a  progressive 
loss  of  comprehension  of  the  environment,  so  that  there  is  no  adequate 
grasp  of  the  present  at  all. 

With  this  mental  failure  goes  a  corresponding  change  on  the  physical 
side.  The  signs  of  age  are  evident  in  the  wasted  muscles,  the  wrinkled, 
inelastic  skin,  gray  hair,  the  raucous  voice,  arcus  senilis,  senile  cuta- 
neous affections,  and  signs  of  arteriosclerosis  in  the  superficial  arteries. 
In  this  condition  it  should  be  remembered  that  the  condition  of  the 
palpable  arteries  may  not  indicate  at  all  the  condition  of  the  cerebral 
vessels.  The  superficial  vessels  may  show  marked  arteriosclerotic 
changes,  while  the  cerebral  vessels  are  in  relatively  good  condition, 
or,  on  the  contrary,  the  cerebral  vessels  may  be  seriously  affected  in 
a  person  whose  radials  are  comparatively  soft  and  whose  temporals 
are  not  noticeably  tortuous. 

Upon  this  background  of  dementia  there  may  appear  the  usual 
pictures — excitements,  depressions,  paranoid  states,  stuporous  states, 
and  confusions.  All  of  these  conditions,  however,  must  be  recon- 
structed in  psychological  terms  to  receive  any  explanation  at  all, 
and  it  is  not  difficult  oftentimes  to  make  out  some  logical  reason  for 
the  particular  type  of  delusional  formation. 

In  the  senile  dement  and  in  the  delusions  of  this  period  there  is 
noticed  an  apparent  indifference  in  the  emotional  sphere,  an  emotional 
poverty.  Persecutory  delusions,  delusions  of  infidelity,  hypochon- 
driacal delusions  take  on  grotesque  forms,  and  their  expression  is 
not  accompanied  by  an  adequate  affect.  A  patient  tells  of  severe 
injuries,  of  having  been  shot,  while  perhaps  smiling;  another  patient 
tells  a  long  and  pitiful  story  about  her  affairs  and  about  a  claim  she 
has  against  the  government,  but  tells  it  in  a  stereotyped,  matter-of- 
fact  way  without  the  expression  of  any  feeling.  This  condition  is 
usually  described  as  one  of  emotional  blunting,  emotional  deteriora- 
tion. Bleuler,^  however,  lays  great  stress  upon  what  he  believes  to  be 
the  fact,  namely,  that  there  is  no  lack  of  capacity  for  feeling,  but  that 
the  affectivity  is  only  disturbed  secondarily,  that  patients  with  organic 
brain  disease  fail  to  get  a  sufficiently  clear  idea  and  therefore  do  not 
react  adequately  in  the  emotional  sphere.     Wherever  it  is  possible 

1  Affectivity,  Suggestibility,  Paranoia.  Translated  by  Chas.  Ricksher,  New  York 
State  Hospital  Bulletin,  February  15,  1912. 


748     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

to  get  a  sufficient  comprehension  of  the  situation  the  emotion  of  the 
proper  quahty  is  manifested  and  with  commensurate  intensity. 

Simple  Senile  Deterioration. — This  condition  of  gradually  progressing 
dementia  without  marked  psychotic  disturbances  is  designated  as 
simple  senile  deterioration. 

The  senile  dement  is  apt  to  be  restless  and  suffer  from  insomnia, 
reversing  the  time  of  day  and  sleeping  perhaps  in  the  daytime  and 
lying  awake  at  night,  perhaps  wandering  about  the  house  at  night 
in  a  more  or  less  disoriented  condition.  This  tendency  to  disorien- 
tation, without  the  development  of  delusions  occurs  in  the  course  of 
the  progressive  deterioration,  and  it  is  where  disturbances  of  the 
sensorium  are  marked  and  confusion  becomes  very  much  more  in 
evidence  than  we  have  in  the  condition  of  senile  delirium.  This 
confusion,  however,  may  be  only  transitory  and  is  quite  character- 
istically in  evidence  in  the  intervals  between  waking  and  sleeping. 

Senile  Delirium, — ^This  is  merely  a  form  of  senile  deterioration  in 
which  confusion  dominates  the  picture  and  in  which  usually  disorders 
of  the  sensorium  are  also  markedly  in  evidence.  Patients  are  highly 
disoriented,  they  do  not  know  where  they  are,  or  what  time  of  day 
it  is,  do  not  know  whether  they  have  just  had  their  dinner  or  whether 
it  is  time  to  go  to  bed  or  get  up,  are  frequently  quite  active  and  irri- 
table, and  are  apt  to  die  from  exhaustion.  The  delirium  may  take 
the  form  of  an  occupation  delirium. 

Presbyophrenia. — A  certain  proportion  of  these  patients  resemble 
very  closely  the  Korsakow's  psychosis,  being  disoriented  as  to  time 
and  place  and  supplying  memory  defects  by  fabrications.  One  old 
man,  for  example,  who  was  so  feeble  he  could  hardly  stand,  relates 
that  he  had  been  working  for  a  man,  making  some  sort  of  a  wire  affair 
for  the  past  seven  months. 

Course. — ^The  course  of  senile  dementia  in  its  various  forms  is  a 
progressive  one.  The  patients  tend  to  become  profoundly  demented, 
wholly  disoriented,  and  die  naturally  of  marasmus.  Where  the  change 
is  somewhat  more  acute,  delirious  or  confusional  episodes  may  be  in 
evidence  from  time  to  time,  and  of  course  if  the  excitement  is  at  all 
prolonged  exhaustion  is  the  result.  The  patients  are  naturally  suscep- 
tible to  intercurrent  affections,  particularly  pneumonia,  nephritis  and 
cystitis,  and  many  of  them  die  in  delirium,  the  result  of  a  terminal 
infection. 

Diagnosis. — Paranoid  conditions  may  resemble  paranoid  states  of 
earlier  life,  but  occurring  in  the  senium,  usually  show  evidences  of 
organic  brain  disease  and  marked  mental  deterioration. 

Some  of  the  patients  also  show  similarities  to  dementia  precox, 
especially  those  that  develop  negativistic  reactions.  Just  what  rela- 
tion these  cases  have  to  the  precox  of  earlier  life  is  not  fully  known. 
The  marked  mental  dilapidation  and  the  period  of  life  at  which  they 
develop  usually  give  the  clue  to  the  diagnosis. 

A  few  cases  show  a  mixed  symptomatology,  presenting  the  picture 


PRESBYOPHRENIA 


749 


of  senile  deterioration  on  the  one  hand,  together  with  the  various 
symptoms  of  arteriosclerotic  disease  with  focal  lesions  on  the  other 
hand.  Here  are  found  all  sorts  of  mixtures  of  pupillary  disturbances, 
disturbances  of  speech,  and  various  forms  of  paralysis. 

Certain  borderland  conditions  show  marked  emotional  states  of 
either  depression  or  excitement  which  dominate  the  picture  for  the 
time  being.  These  cases  show  beneath  the  emotional  state  the  presence 
of  a  defect  which  shows  the  basal  disorder. 

The  similarity  between  presbyophrenia  and  Korsakoff's  disease  is 
very  considerable.  Nouet^  has  recently  made  a  careful  study  of  the 
two  conditions  and  has  set  down  the  following  differential  criteria: 

Korsakow's  psychosis  affects  persons  particularly  of  adult  age; 
presbyophrenia,  on  the  contrary,  belongs  to  the  period  of  old  age, 
the  age  of  predilection  oscillating  about  seventy  years.  Presbyo- 
phrenia, it  is  known,  is  quite  uniquely  an  affection  of  women,  while 
Korsakow's  psychosis  is  far  from  being  rare  among  men.    The  humor 


Fig.  314. — Presbyophrenic  facies. 

of  the  presbyophrenic  is  always  gay,  euphoric  and  satisfied,  quite 
different  from  the  psychopolyneuritic,  who  is  ordinarily  depressed  or 
apathetic,  and  whose  face  preserves  an  invariable  immobility.  The 
traits  of  the  presbyophrenic  are  extreme  mobility,  they  laugh,  make 
grimaces  in  which  the  mimic  muscles  participate.  The  facies  of  the 
psychopolyneuritics,  however,  are  always  dull  and  without  expression. 
Loquacity  is  a  symptom  scarcely  ever  lacking  in  presbyophrenia; 
the  patients  talk  without  stopping  about  everything,  with  equal 
volubility.  This  symptom  is  lacking  in  Korsakow's  psychosis.  The 
presbyophrenic  is  polite,  amiable  and  cordial,  characteristics  which 
one  seeks  in  vain  among  the  psychopolyneuritics.  Disorders  of  con- 
sciousness are  much  more  marked  in  the  presbyophrenic.  These 
patients  have  no  understanding  of  their  state  of  illness  and  content 
themselves  with  laughing  when  one  asks  a  question  relative  to  their 


*  Presbyophrenie  de  Wernicke  et  les  Psychopolyneurites,  L'Encephale,  February  10, 
1911. 


750     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

physical  or  mental  health.  On  the  contrary  the  psychopolyneuritic 
gives  a  fairly  good  account  of  himself  and  of  his  position,  and  is  the 
first  to  lament  his  situation.  The  judgment  of  these  patients,  even  in 
the  chronic  forms,  is  less  noticeably  affected,  and  their  degree  of 
intellectual  enfeeblement  less  marked.  The  amnesia,  finally,  is  more 
profound  in  the  presbyophrenic,  and  besides  in  this  affection  the  patient 
does  not  possess  at  all  a  knowledge  of  this  amnesia — the  inverse  of 
what  one  observes  in  the  chronic  forms  of  Korsakow's  disease,  where 
the  subjects  speak  spontaneously  to  their  interlocutors  of  the  profound 
troubles  of  their  memory.  Presbyophrenia  is  rare  in  its  typical  forms, 
but  common  in  the  formes  frustes.  Where  the  symptoms  only  approach, 
or  where  certain  of  them  are  lacking,  the  presbyophrenia  has,  perhaps, 
the  distinctive  characteristics  of  arteriosclerosis,  which  are  seen 
much  more  among  these  patients  than  among  simple  senile  dements. 
Patients  die  nearly  always  of  cerebral  hemorrhage,  and  their  nervous 
centers  present  at  autopsy  pronounced  atheromatous  lesions. 

Pathology. — Grossly  the  brain  shows  signs  of  atrophy  and  is  decreased 
in  weight,  the  bones  of  the  skull  are  thinned,  sometimes  in  well-defined 
regions,  particularly  the  temporal  regions.  There  is  a  compensatory 
external  hydrocephalus  as  a  result  of  the  atrophy  of  the  brain.  The 
convolutions  are  shrunken  and  the  fissures  correspondingly  widened. 
The  atrophy  is  not  uniformly  distributed  and  may  be  very  much  more 
marked  in  some  areas  than  others.  Arteriosclerosis  is  a  frequent 
finding,  but  is  not  a  necessary  part  of  the  picture.  In  fact  the  two 
processes  are  quite  distinct  in  every  way,  although  frequently  asso- 
ciated. When  arteriosclerosis  is  present  there  may  of  course  be  found 
its  results  in  such  lesions  as  softenings. 

The  architectonic  of  the  cortex  is  greatly  disturbed.  The  nerve 
cells  show  advanced  degeneration  with  large  quantities  of  degenerative 
products  of  a  lipoid  nature  within  them,  amounting  to  a  severe  grade 
of  fatty  degeneration.  A  very  characteristic  picture  are  the  basket 
formations  about  the  nerve  cells.  The  neurofibrils  appear  to  be 
thickened  and  produce  whirls  and  loops  about  the  nerve  cell.  It  is 
thought  by  some  that  these  basket  formations  are  due  to  incrustations 
upon  the  neurofibrils  of  perhaps  neurogliar  origin.  The  condition  is 
particularly  well  seen  in  presbyophrenia. 

The  miliary  plaques  are  perhaps  the  most  distinctly  pathological 
findings  in  senile  dementia.  In  fact  they  are  distinctive  as  much  as 
any  pathological  finding  can  be  distinctive  of  any  one  condition. 
Their  presence  in  considerable  numbers  practically  makes  the  diag- 
nosis of  senile  dementia,  while  their  absence  or  extreme  rarity  would 
negative  such  a  diagnosis.  They  are  small  spots  of  necrosis  which 
are  not  dependent  upon  softenings  and  are  in  no  way  associated  with 
the  bloodvessels. 

There  is  a  great  deal  of  neurogliar  proliferation  throughout  the 
brain  and  marked  evidences  of  disintegration  and  the  presence  of 
scavenger  cells  and  the  like. 


ALZHEIMER'S  DISEASE  751 

Aside  from  these  changes  the  usual  changes  of  this  period  of  Hfe 
are  found,  particularly  in  the  heart,  the  kidneys,  and  other  internal 
organs. 

Treatment. — The  mild  cases,  especially  those  that  maintain  their 
orientation  fairly  well,  can  be  cared  for  at  home.  Those  with  marked 
confusion,  especially  with  a  tendency  to  wandering,  need  an  attendant, 
to  be  with  them.  There  is  danger  of  their  becoming  lost  and  coming 
to  grief,  or  if  they  wander  about  the  house  at  night  they  are  apt  to 
meet  with  some  accident,  more  often  to  fall  down  stairs  and  sustain 
fractures.  Patients  who  are  very  resistive,  present  surgical  troubles, 
are  filthy  in  habits,  or  show  a  tendency  to  commit  sexual  offences, 
should  be  cared  for  in  an  institution. 

As  regards  the  more  special  treatment,  little  is  to  be  said.  Hygienic 
surroundings,  a  simple  diet,  looking  after  the  emunctories,  and  if 
insomnia  is  present  the  occasional  exhibition  of  a  hypnotic  constitutes 
about  all  there  is  to  be  done.  In  this  class  of  cases,  more  perhaps  than 
in  any  other,  is  the  use  of  alcohol  as  a  hypnotic  indicated.  A  little 
whisky  and  hot  water,  or  a  glass  of  beer  or  ale  acts  very  nicely.  It 
should  be  given,  however,  strictly  under  medical  authority  and  super- 
vision, as  these  patients  are  apt  to  be  susceptible  to  its  influences. 
In  the  earlier  stages  of  the  disease  potassium  iodide  is  the  drug  par 
excellence  for  its  general  alterative  properties  and  its  effect  on  the 
arterial  tension. 

As  soon  as  evidences  of  mental  deterioration  appear  relief  from 
business  worries,  cares,  responsibilities,  and  mental  stresses  of  all  sorts 
is  indicated,  with  the  general  hope  of  limiting  disintegration  as  far  as 
possible.  Whether  this  is  of  value  or  not  is  pretty  difficult  to  state, 
for  it  must  be  remembered  that  there  appear  to  be  certain  hereditary 
tendencies  involved  even  in  this  condition  and  that  arteriosclerotic 
disease  and  senile  dementia  both  appear  to  be  more  prevalent  in 
certain  families. 

Alzheimer's  Disease. — -This  disease  was  first  described  by  Alzheimer 
in  1906  and  since  that  time  a  number  of  cases  have  been  reported. 
The  disease  occurs  usually  in  the  fifth  decade,  although  cases  have 
been  reported  in  the  early  part  of  the  fourth  decade  and  as  late  as  the 
beginning  of  the  seventh.  The  symptomatology  is  one  of  a  gradually, 
often  of  a  rather  rapidly  progressing  dementia,  interrupted  perhaps 
with  episodes  of  a  certain  amount  of  excitement  and  anxiet}'.  A 
rather  rapidly  progressing  dementia  in  a  man  of  about  forty,  or  a 
little  over,  is  in  itself  a  rather  unusual  picture,  and  in  the  absence 
of  signs  of  brain  tumor  or  syphilis,  Alzheimer's  disease  should  be 
thought  of.  The  dementia  is  marked  by  a  considerable  degree  of 
disorientation.  The  symptoms  otherwise  are  very  largely  neurological 
and  particularly  focalized  about  the  function  of  speech.  Various 
aphasic  and  apraxic  symptoms  are  prominently  in  evidence.  Para- 
phasia and  asymbolia  are  quite  frequent.  There  may  also  be  spasticity 
and  convulsions,  although  there  are  never  well-marked  paralyses. 


752     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

The  pathology  of  this  condition  throws  almost  the  only  light  upon 
its  nature.  There  is  marked  and  extensive  degeneration  of  the  nerve 
cells  with  disturbances  of  cortical  architectonic  and  the  presence  of 
large  quantities  of  disintegration  products,  while  there  are  found 
numbers  of  basket  formations  and  typical  miliary  plaques.  The  con- 
dition of  the  bloodvessels  is  quite  normal.  The  pathological  picture 
thus  resembles  closely  that  found  in  senile  dementia.  In  general, 
the  disease  is  considered  as  a  presenile  dementia,  although  some  believe 
it  to  be  a  distinctive  disease. 

The  presence  of  this  disease,  if  it  be  considered  a  presenile  dementia, 
offers  another  one  of  those  warnings  to  us  not  to  be  too  dogmatic. 
It  would  appear  that  the  senium  is  by  no  means  a  clearly  defined 
period  of  life,  and  that  the  pathological  changes  which  are  usually 
thought  of  as  dependent  upon  old  age,  may  occur  within  wide  limits 
and  perhaps  represent  failure  of  special  tissue  resistances. 

Arteriosclerotic  Psychoses. — These  psychoses  are  dependent,  on 
their  physical  side,  upon  arteriosclerotic  changes  in  the  cerebral  blood- 
vessels, and  this  condition  is  due  in  turn  to  the  general  causes  which 
produce  arteriosclerosis.  In  the  main  the  two  factors  are  chronic 
toxemia  and  high  blood-pressure.  It  must  be  borne  in  mind,  how- 
ever, that  an  advanced  degree  of  arteriosclerosis  may  exist,  particu- 
larly in  the  peripheral  vessels,  and  the  cerebral  vessels  retain  their 
elasticity,  while  on  the  contrary  the  cerebral  vessels  may  be  severely 
sclerosed  while  the  peripheral,  palpable  vessels  show  little  change. 
Arteriosclerosis  is  essentially  a  regional  disease. 

The  psychoses  of  arteriosclerosis  form  another  one  of  the  connecting 
links  which  join  the  period  of  involution  and  the  senium.  Many  of 
the  involution  psychoses  merge  into  arteriosclerotic  deterioration, 
and  arteriosclerosis  is  frequently  combined  with  the  changes  incident 
to  the  senium. 

Aside  from  the  usual  causes  of  arteriosclerosis  there  would  seem  to 
be  certain  hereditary  factors  at  play.  Certain  families  show  a  high 
incidence  of  death  dependent  upon  arteriosclerotic  disease. 

There  are  four  fairly  well-defined  varieties  of  this  disease  based 
upon  both  clinical  and  pathological  findings  as  follows: 

1.  Arteriosclerotic  Brain  Atrophy. — This  occurs  in  two  forms:  a  mild 
form  with  severe  arterial  sclerosis  but  an  absence  of  focal  brain  lesions. 
The  symptoms  are  easy  fatigue,  slight  failure  of  memory,  dizziness 
and  headache.  The  severe  type  may  resemble  the  mild  at  first  but  is 
progressive,  leads  to  profound  dementia  and  presents  in  its  course 
apoplectiform  and  epileptiform  attacks  and  focal  symptoms. 

2.  Subcortical  Encephalitis  (Binswanger).  —  In  this  condition  the 
white  matter  is  largely  involved  as  a  result  of  disease  of  the  long 
medullary  arteries.  Apoplectiform  and  epileptiform  attacks  occur 
and  also  transitory  attacks  of  confusion,  aphasia  and  paresis,  dis- 
turbances suggesting  focal  lesions.  Focal  lesions  are  not  found  exten- 
sively but  areas  of  softening  often  occur  in  the  basal  ganglia. 


ARTERIOSCLEROTIC  PSYCHOSES  753 

3.  Perivascular  Gliosis. — In  this  condition  there  is  a  disappearance 
of  nervous  elements  about  the  diseased  vessels  and  replacement  by 
neuroglia. 

4.  Senile  Cortical  Devastation.— Here  extensive  destruction  of 
cortical  areas  in  the  vascular  territories  of  the  diseased  vessels  is 
found.  In  this  condition  the  arteriosclerosis  is  localized  largely  in  the 
small  cortical  vessels  which  come  off  from  the  pia.  The  basal  vessels 
remain  relatively  normal. 

Pick  has  very  thoroughly  described  certain  large  atrophies  involving 
whole  lobes  or  portions  of  lobes.  The  occipital  lobes  may  be  involved, 
producing  blindness,  or  the  temporal,  producing  deafness,  for  example. 
The  atrophy,  however,  does  not  always  follow  a  vascular  area,  and  so, 
while  it  is  generally  supposed  to  be  due  to  arteriosclerotic  disturbances 
in  the  irrigation  of  these  territories,  the  cause  is  not  always  altogether 
clear. 

Symptoms. — In  the  main  the  symptomatology  of  cerebral  arterio- 
sclerosis is  one  of  gradually  progressive  mental  deterioration,  to 
which  are  added  the  evidences  of  focal  lesions  which  are  the  results 
of  thrombotic  softenings.     (See  Chapter  on  Hemorrhage.) 

The  prodromal  disturbances  of  the  arteriosclerotic  psychoses  are 
very  apt  to  extend  over  a  considerable  period  of  time,  and  manifest 
themselves  in  the  main  as  nervousness  and  irritability,  with  headache, 
dizziness,  insomnia,  associated  of  course  with  the  special  signs  of 
the  vascular  disease,  more  particularly  as  a  rule,  high  blood-pressure. 
With  this  series  of  symptoms  there  may  be  of  course  associated  a 
certain  amount  of  deafness,  with  sclerosis  of  the  drum  membrane, 
cardiac  attacks  with  the  Stokes-Adams  syndrome  and  evidences  of 
interstitial  nephritis.  Quite  frequently,  too,  these  patients  show  the 
signs  of  arteriosclerosis  of  the  vessels  of  the  spinal  cord,  with  perhaps 
some  sclerosis  of  the  palpable  vessels.  On  the  mental  side  the  patient 
may  have  a  feeling  of  growing  inefficiency,  at  least  his  work  shows  a 
falling  off  in  efficiency,  which  characteristically  manifests  itself  at  first 
in  a  failure  to  do  the  creative  things.  Pick  has  called  particular 
attention  on  the  emotional  side  to  a  lack  in  the  finer  modulations  of 
the  emotions.  Bleuler's  suggestion  must  be  remembered,  that  patients 
with  organic  brain  disease  fail  to  show  a  natural  emotional  reaction, 
not  because  of  any  defect  of  emotion,  but  because  of  a  lack  of  grasp 
of  the  situation,  and  when  the  situation  is  fully  appreciated  an  adequate 
emotional  response  issues. 

These  prodromal  symptoms  gradually  merge  into  symptoms  of 
greater  severity  and  may  be  punctuated  from  time  to  time  by  attacks 
of  excitement  or  of  depression,  and  delusions  may  develop  which 
are  characteristically  of  the  paranoid  type.  The  following  case  illus- 
trates this  paranoid  trend  very  well :  A  man  who  had  been  a  successful 
business  man  in  his  younger  days  began  to  fall  off  in  efficiency  as  he 
entered  the  arteriosclerotic  period  of  life,  and  finally  entered  one  of 
the  Soldiers'  Homes  as  he  was  unable  to  adequately  support  himself. 
48 


754     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

Here,  because  of  his  training  as  an  accountant,  he  was  employed  in 
the  office.  While  employed  here  the  characteristic  series  of  paranoid 
ideas  developed.  In  the  first  place  he  developed  exalted  ideas  of  his 
own  ability  which  were  distinctly  defensive  in  character,  compen- 
sating him  for  the  real  fact,  his  failing  ability.  Alongside  of  these 
exaggerated  ideas  he  had  delusions  of  a  persecutory  character.  He 
was  interfered  with  by  those  about  him  who  were  envious  of  him 
because  he  had  secured  such  a  good  position  in  the  Governor's  office. 
This  is  another  defense  reaction  in  the  opposite  direction  and  serves 
to  explain  to  him  how  a  really  efficient  man  after  all  can  turn  out  such 
poor  work.  It  is  not  his  fault,  but  the  fault  of  those  about  him,  and 
so  he  is  again  saved  from  the  realization  of  his  failings.  Removed  from 
this  situation  and  taken  to  a  hospital  where  he  could  no  longer  indulge 
in  alcohol,  which  had  been  a  factor  in  bringing  about  this  condition, 
he  recovered  from  these  distinct  psychotic  manifestations,  but  without, 
however,  a  full  insight  into  what  his  condition  had  been.  This  lack 
of  insight  probably  had  its  basis  in  the  organic  changes.  The  man  was 
no  longer  capable  of  adjustment,  except  within  narrow  limits,  and 
while  removal  from  the  painful  conditions  relieved  the  situation,  he 
was  unable  to  fully  understand  it. 

This  case  shows  very  well  how  even  in  a  psychosis  dependent  upon 
organic  brain  disease  the  mental  symptoms  as  such  must  receive  a 
psychological  interpretation. 

From  time  to  time  these  patients  show  periods  of  confusion  and 
bewilderment,  with  disorientation.  These  periods  may  be  of  consider- 
able duration  and  some  of  them  at  least  are  dependent  upon  thrombotic 
attacks  which,  when  they  do  not  occur  in  the  motor  area,  are  easily 
overlooked.  Even  though  they  do  occur  in  the  motor  area  the  dis- 
turbance of  consciousness  may  not  be  very  great,  or  at  least  there 
is  nothing  comparable  to  the  unconsciousness  and  coma  of  cerebral 
hemorrhage;  there  is,  perhaps,  only  a  slight  confusion,  and  the  paraly- 
sis, if  it  exists,  is  only  very  slight  and  is  often  explained  by  the  family 
as  the  result  of  some  inconsequential  cause.  Then  again  the  patient 
may  be  so  blunted  mentally  as  not  to  complain  of  a  shght  impairment 
of  function. 

These  patients  are  especially  susceptible  to  alcohol  and  become 
easily  very  badly  confused  from  small  quantities. 

One  of  the  characteristic  features  with  regard  to  this  group  of 
psychoses  is  that  the  so-called  "nucleus  of  the  personality"  is  well 
preserved.  The  patient  preserves  all  of  the  outward  appearances  of 
his  old  self  until  the  mental  dilapidation  has  reached  an  extreme 
degree.  There  is  not,  as  a  rule,  that  marked  "change"  in  the  indi- 
vidual that  is  seen  in  some  of  the  psychoses. 

The  areas  of  softening  produce  focal  lesions  and  these  focal  lesions 
are  most  prominent  in  the  motor  areas,  producing  various  upper 
motor  neuron  paralyses,  and  in  the  speech  areas  producing  various 
forms  of  aphasia  and  apraxia.    The  marked  focal  lesions,  particularly 


ARTERIOSCLEROTIC  PS  YC HOSES 


755 


those  which  lead  to  well-defined  speech  disturbances  hasten  very 
greatly  the  dementia.  An  aphasia  which  puts  the  patient  out  of  actual 
touch  with  his  fellows  hastens  the  tendency  to  mental  deterioration 
in  this  class  of  patients  who  are  no  longer  fluid,  but  are  well  along 
on  the  down-hill  path  of  life.  They  need  every  stimulus  of  mental 
activity  to  even  hold  their  own,  and  as  soon  as  an  important  function 
like  speech  is  destroyed  they  are  quite  apt  to  lapse  promptly  into  a 
serious  deterioration. 

It  is  in  this  class  of  patients  that,  as  a  result  of  the  focal  lesions, 
epileptiform  attacks  develop  late  in  life — the  so-called  "late  epilepsies." 


F 

P 

,,, 

'"""^              'gB»' 

^ 

1 

1 

*K 

1 

f 

^^^^^H 

K 

,  j^,_ 

Mr-^M 

L  |A|      'f J| 

^sM2'^ 

9 

^HHHHj^HHH 

Hhh 

^M 

^^^^HHHI 

HH 

*'■  '^l^'^'^^il^^^^^ 

„j6. 

^V 

^^^^^PfwS 

^BR* 

1^--,- 

m^~^. 

'■  \_^^l 

^^^Ik.       -^^ 

K. 

^^'^^^■i 

usS^" 

\^^^^M 

^^^^Hk^^^a 

i^l^^H 

^^^^^^^^^^^^^^^^^H 

^^Hr^wji 

•^       JL      -**'"^^| 

^^SskSSB^^  -^^aB|H|K!^^^ 

'^r^^^^^S' 

^^^^^^^^^1 

1 

1 

^ -"^^TS^S^^^ 

1 

Fig.  315. — Extensive  thrombotic  softening,  the  result  of  arteriosclerosis.  Patient  aged 
eighty-three  years.  There  were  also  softenings  on  the  right  side  in  the  lower  portion 
of  the  cuneus,  and  upper  portions  of  the  lingual  and  fusiform  lobules.  There  is  general 
atrophy  of  the  convolutions. 


Their  significance  in  the  main  is  in  pointing  to  the  focal  disorder, 
and  as  an  indication  that  the  patient  needs  careful  oversight. 

Pathology. — The  pathology  shows  the  arteriosclerotic  process  in 
the  cerebral  vessels  in  various  stages  of  progress.  There  may  be  miliary 
aneurisms  of  the  smaller  vessels  and  other  vessels  may  be  completely 
occluded  with  resulting  areas  of  softening.  These  areas  of  softening 
usually  show  entire  disintegration  of  the  nervous  elements  with 
large  numbers  of  scavenger  elements  about.  The  nerve  cells  show 
various  grades  of  degenerative  change  dependent  upon  deficient  nutri- 
tion as  a  result  of  decrease  in  size  of  the  lumen  of  the  vessels.    There 


756     PRESENILE,  SENILE,  AND  ARTERIOSCLEROTIC  PSYCHOSES 

is  neurogliar  overgrowth  about  the  vessels  and  in  the  degenerated 
territories. 

Diagnosis. — The  disease  most  apt  to  be  mistaken  for  the  arterio- 
sclerotic psychoses  are  paresis  and  the  psychoses  of  cerebral  syphilis, 
which  should  be  considered  together  now  that  the  etiology  of  paresis 
has  been  so  thoroughly  established.  The  reason  for  the  possibilities 
of  mistake  is  dependent  upon  the  fact  that  both  processes  are  more  or 
less  diffuse,  producing  a  progressive  mental  deterioration,  and  that 
both  processes  tend  to  be  more  severe  at  certain  points  and  thus  pro- 
duce  focal   symptoms.     Syphilitic   and   metasyphilitic  disturbances 


Fig.  316. — Widely  distributed  arteriosclerotic  softenings;  patient  aged  seventy-two 
years.  There  were  also  numerous  softenings  in  the  basal  ganglia.  Brain  greatly 
shrunken.  It  can  be  seen  from  the  distribution  of  these  lesions  how  the  clinical  picture 
might  simulate  paresis. 


occur  as  a  rule  at  a  much  earlier  age  than  the  arteriosclerotic,  generally 
not  later  than  the  fourth  decade,  while  the  arteriosclerotic  period  is 
not  entered  until  the  fifth  decade.  When  an  arteriosclerotic  has  had 
syphilis  also  the  differentiation  becomes  more  difficult.  The  Was- 
sermann  of  the  cerebrospinal  fluid,  however,  would  be  negative,  while 
with  large  areas  of  softening  on  the  surface  of  the  cerebrum  there 
would  be  considerable  evidence  of  disintegration  products  in  the 
cerebrospinal  fluid.  On  the  mental  side  the  preservation  of  the 
"nucleus  of  the  personality"  is  much  more  in  evidence  than  in  paresis. 
Treatment. — In  a  general  way  the  treatment  should  be  prophylactic 
as  far  as  possible.    With  the  first  symptoms  of  prolonged  and  intract- 


ARTERIOSCLEROTIC  PSYCHOSES  757 

able  high  blood-pressure  the  individual  should  be  removed  from  the 
influences  of  physical  and  mental  stress,  and  the  usual  means  should 
be  employed  to  keep  the  blood-pressure  down  and  to  reduce  or  do 
away  with  any  toxemic  condition  present.  A  carefully  regulated  diet 
from  which  alcohol  and  tobacco  are  excluded,  hydrotherapy  intelli- 
gently applied,  possibly  a  visit  to  some  watering  place,  and  in  some 
instances  a  visit  to  a  somewhat  higher  altitude,  with  moderate  out- 
door exercise,  and  the  exhibition  from  time  to  time  of  drugs  to  reduce 
the  pressure  and,  especially  where  there  are  kidney  complications, 
the  drinking  of  considerable  quantities  of  mildly  alkaline  water  are 
in  general  the  things  to  be  depended  upon.  Insomnia  has  to  be  dealt 
with,  and  in  cases  where  the  patient  is  under  absolute  control  a  small 
amount  of  alcohol  at  night  will  sometimes  produce  the  desired  result. 
Unless  the  patient  is  under  absolute  control,  however,  it  is  best  to  give 
hot  milk,  or  such  simple  hypnotics  as  veronal. 


CHAPTER  XXV. 

IDIOCY,  IMBECILITY,  FEEBLE-MINDEDNESS,  AND 
CHARACTEROLOGICAL  DEFECT  GROUPS. 

In  drawing  a  distinction  between  dementia  and  idiocy  Esquirol 
said:  "The  demented  man  is  deprived  of  the  good  that  he  formerly 
enjoyed;  he  is  a  rich  man  become  poor;  the  idiot  has  always  lived  in 
misfortune  and  poverty."  In  other  words  the  idiot,  the  imbecile,  and 
the  feeble-minded  lack  something;  the  psychotics  are  suffering  from  a 
disorder  of  that  which  they  possess. 

This  definition,  so  admirably  worded  by  Esquirol,  adequately  ex- 
pressed the  distinction  between  the  insane  and  the  idiot  and  the 
imbecile  according  to  the  knowledge  of  his  day,  and  has  been  used 
continuously  since  to  express  such  distinction.  It  can  no  longer  be 
said  to  be  a  valuable  formula  except  it  be  used  with  many  reservations 
and  explanations. 

In  the  first  place,  from  the  standpoint  of  this  book  there  is  no  such 
thing  as  insanity,  at  least  in  a  medical  sense,  as  Esquirol  used  that 
term  and  as  other  psychiatrists  in  the  past  and  the  present  have  used  it. 
Insanity  is  purely  a  legal  and  sociological  concept,  and  as  such  does 
not  imply  anything  more  than  the  judgment  of  a  man's  fellows  on  the 
desirability  of  having  him  live  in  the  community.  Defectiveness,  under 
which  term  the  various  grades  of  idiocy,  imbecility,  and  feeble-minded- 
ness  are  included,  is  quite  as  generic  a  term,  and  while  it  refers  to  a 
condition  the  fundamental  characteristic  of  which  is  lack  of  develop- 
ment, it  includes  a  great  variety  of  states,  and  in  its  application  is  a 
relative  term  only. 

It  is  quite  as  illogical  to  group  all  defectives  together  and  endeavor 
to  draw  a  conclusion  from  their  study  as  a  whole,  as  it  is  to  group  all 
of  the  so-called  insane  together  and  endeavor  to  draw  a  conclusion 
from  the  study  of  all  of  them.  There  is  obviously  very  little  similarity 
between  the  cretin  and  the  defective  as  a  result  of  cerebral  hemorrhage 
during  a  prolonged  and  difficult  labor,  and  therefore  any  principles 
or  conclusions  which  are  reached  as  regards  the  whole  defective  class 
without  an  adequate  appreciation  of  the  multitude  of  different  con- 
ditions that  are  found  under  this  generic  caption  must  be  avoided. 
It  must  be  remembered,  too,  that  with  any  defective  patient  it  is 
possible  to  have  associated  a  psychosis,  so  that  the  picture  may  be  still 
more  complicated.  Therefore  in  other  parts  of  this  book  various  forms 
of  what  are  grouped  in  this  chapter  are  treated  of  from  an  etiological 
standpoint. 


CLASSIFICATION  759 

In  considering  the  etiology  of  various  defect  states  the  important 
general  principle  that  should  be  borne  in  mind  is  the  location  of  the 
defect;  first,  as  to  whether  it  is  in  the  germ  plasm  or  acquired,  and 
second,  as  to  the  time  of  development  of  the  individual  when  it  became 
operative.  It  is  only  those  defects  which  are  the  result  of  a  lack  of 
some  element  in  the  germ  plasm  that  are  truly  of  an  hereditary  nature. 

The  process  of  development  may  be  interrupted  at  any  time  from 
the  beginning  of  the  growth  of  the  child  in  the  uterus  to  the  attain- 
ment of  its  complete  adult  development,  and  as  this  interruption  may 
take  place  during  intra-uterine  life  it  is  in  such  instances  that  it  is 
important  to  make  the  distinction  between  a  truly  inherited  and  an 
acquired  defect.  In  both  instances  the  defect  is  congenital,  that  is, 
exists  from  birth. 

Disease  or  injury  may  affect  the  child  during  intra-uterine  life  or 
during  the  process  of  birth  or  after  birth  and  during  the  processes 
of  extra-uterine  development.  Previous  to  birth,  illness  or  injury  to 
the  mother  are  the  common  etiological  factors;  during  birth  asphyxia- 
tion and  injury  by  the  forceps  are  common  causes,  while  after  birth 
the  infectious  diseases  and  direct  injuries  enter  largely  into  the  etiology. 
Finally,  there  are  relative  conditions  of  defect  which  are  due  to  lack 
of  the  opportunity  to  develop,  such  defect,  for  example,  as  results  from 
the  deprivation  of  the  important  sense  organs,  as  the  eyes  and  ears. 
If  the  patient  be  born  blind  and  deaf,  under  ordinary  circumstances 
he  will  be  very  defective  mentally,  because  he  has  not  the  opportunities 
for  learning  which  the  ordinary  child  has.  On  a  still  different  plane 
relative  defect  due  to  sordid  and  unsanitary  conditions  and  lack  of 
educational  facilities  is  found.  Children  are  ignorant  who  have  no 
opportunity  to  go  to  school  and  learn,  and  may  even  lack  the  ordinary 
brightness  that  comes  about  spontaneously  if  they  have  lived  under 
insanitary  conditions  that  impaired  their  general  health  and  energies, 
especially  if  these  conditions  be  complicated  by  the  presence  in  the 
child  of  some  such  debilitating  factor  as  infected  tonsils,  adenoids, 
high  degree  of  myopia,  otitis  media  with  deafness,  and  such  other  like 
things  which  impair  the  general  health  and  vigor  of  the  child  and 
interfere  with  the  patency  of  the  avenues  through  which  he  gains  his 
information  of  the  outer  world.  This  general  enfeeblement  of  the 
child  may  also  be  the  result  of  debilitating  habits,  such  as  masturbation 
and  the  use  of  narcotics  and  alcoholic  drinks,  the  latter  of  which 
especially  are  an  extreme  expression  of  a  vicious  environment. 

Classification. — The  general  group  of  defectives  is  a  very  wide  one 
and  includes  not  only  the  idiots  and  the  imbeciles  and  the  frankly 
feeble-minded,  but  the  still  higher  grades  of  backward  children,  and 
then  a  whole  borderland  group  of  ne'er-do-wells  in  whom  the  defect  is 
much  less  clearly  defined  than  in  the  lower  grades. 

David  Starr  Jordan  has  said  that  "A  good  citizen  is  one  who  can 
take  care  of  himself  and  has  something  left  over  for  the  common 
welfare."     This  saying  represents  in  a  general  way  the  basis  on  which 


760  IDIOCY,   IMBECILITY,  AND  DEFECT  GROUPS 

until  recently  the  classification  of  this  group  has  been  based.  The 
defectives  as  a  class  may  be  said  not  only  not  to  have  anything  left 
over  for  the  common  welfare,  but  only  in  the  highest  grades,  and  then 
under  the  most  favorable  circumstances,  can  the}'^  care  for  themselves. 
The  general  basis  of  classification,  therefore,  has  been  their  educability 
and  their  capacity  to  care  for  themselves,  according  to  some  such 
scheme  as  follows,  for  example: 

Feeble-mindedness. — ^A  condition  of  mental  defectiveness  capable 
of  much  improvement  by  educational  methods.  The  afflicted  individ- 
ual may  ultimately  take  a  place  in  the  world  and  be  self-supporting 
under  favorable  circumstances. 

Imbecility. — A  condition  of  mental  deficiencj''  which  can,  however, 
be  materially  improved  by  training,  but  not  sufficiently  for  the  subject 
to  take  a  place  in  the  world. 

Moral  Imbecility. — A  condition  of  mental  defectiveness  which  is 
shown  predominantly  in  the  absence  of  the  highest  functions,  particu- 
larly the  moral;  capable  of  training  to  a  considerable  degree,  but 
always  a  menace  to  society. 

Idio-imbecility. — A  condition  midway  between  idiocy  and  imbecility. 

Idiocy. — A  condition  of  profound  mental  defectiveness.  The  lower 
grades  are  unteachable,  while  the  higher  may  be  trained  slightly  in 
self-help,  I.  e.,  to  attend  to  the  calls  of  nature. 

More  recently  an  attempt  has  been  made  to  define  with  greater 
accuracy  the  different  grades  of  defect,  and  this  effort  has  taken  the 
form  of  an  attempt  to  correlate  the  psychological  development  of  the 
defective  with  the  psychological  development  of  the  average  child, 
so  that  the  defective  as  a  result  of  this  correlation  is  said  to  correspond 
to  the  development  of  the  average  child  at  such  and  such  an  age.  In 
other  words,  age  has  come  to  have  a  psychological  rather  than  a 
chronological  significance,  and  an  individual  who  may  be  forty  years 
old,  but  who  is  only  developed  to  the  extent  that  an  average  child  is 
developed  at  the  age  of  seven,  is  spoken  of  as  having  the  psychological 
age  of  seven  years. 

Until  the  use  of  this  scheme  of  classification  all  people  were  classified 
among  other  ways,  in  accordance  with  their  chronological  age.  For 
example,  in  this  country  a  person  attains  his  majority  and  can  cast 
his  vote  at  the  age  of  twenty-one.  This  refers  of  course  entirely  to  the 
chronological  age.  It  can  easily  be  seen  that  if  there  are  a  material 
percentage  of  persons  in  the  community  who  are  defectives,  although 
they  may  have  acquired  the  chronological  age  of  twenty-one  they  have 
not  the  mental  development  and  the  judgment  that  it  is  expected 
goes  with  such  an  age,  and  therefore  from  the  psychological  standpoint 
are  not  twenty-one  and  ought  not  on  that  basis  be  permitted  to  vote. 
The  psychological  classification  of  the  age  as  outlined  by  the  Binet- 
Simon  test  is  a  far  more  accurate  way  to  standardize  the  mental 
development  of  the  individual  than  the  chronological  age  method, 
and  although  there  are  objections  which  may  be  urged  against  it  and 


IDIOCY 


761 


although  it  is  concededly  not  a  perfect  method  and  is  at  present 
undergoing  gradual  modifications,  still  it  is  so  much  better  than  the 
chronological  method  that  it  should  be  used  in  its  place,  and  is  by 
far  the  best  standard  which  we  have  for  designating  the  development 
of  the  defective.  The  classification,  according  to  this  method  which 
has  recently  been  adopted  by  the  American  Association  for  the  Study 
of  the  Feeble-minded,  is  as  follows: 


Mental  age. 

Under  one  year 

1  year 

2  years 

3  years 

4  years 

5  years 

6  years 

7  years 

8  years 

9  years 

10  years 

11  years 

12  years 


Capabilities. 

Helpless 

Feeds  self.     Eats  everything 

Eats  discriminatingly 

No  work.     Plays  little 

Tries  to  help 

Only  the  simplest  tasks 

Tasks  of  short  duration.     Washes  dishes 

Little  errands  in  house.     Dusts 

Errands.     Light  work.     Makes  beds 

Heavier  work.  Scrubs,  mends,  lays  bricks, 
cares  for  room  with  simple  furniture 

Good  institution  helpers.     Routine  work 

Fairly  complicated  work  with  only  occa- 
sional oversight 

Uses  machinery.  Cares  for  animals.  No 
supervision.     Cannot  plan 


Class. 

Low 
Middle 
High 
Low 

Middle 

High 
Low 


\  Idiot. 


Imbecile. 


Middle 


High 


>  Moron. 


The  classification,  according  to  the  psychological  age,  while  it  is 
practically  useful  for  defining  the  stage  of  development  which  the 
individual  has  reached,  is  also  of  some  value  in  differentiating  hered- 
itary and  acquired  conditions.  Somewhere  from  65  to  80  per  cent, 
of  defectives  have  feeble-minded  parents,  and  therefore  in  most  of 
them  the  condition  is  hereditary.  In  these  hereditary  cases  it  would 
seem  as  if  the  child  developed  quite  normally  up  to  a  certain  point  and 
then  stopped  rather  suddenly,  and  that  this  stopping  was  a  pretty 
general  one,  so  that  the  development  ceases  at  a  certain  level  without 
many  irregularities.  A  patient,  for  example,  will  test  to  seven  years 
of  age,  will  do  practically  all  of  the  tests  for  seven  years,  and  fail 
completely  with  all  of  the  tests  above  that.  In  other  words,  the  age 
is  very  accurately  seven.  The  defect  is  a  clean-cut  one.  When  this 
condition  of  affairs  is  found  there  are  reasons  to  suppose  that  the 
trouble  lies  in  the  germ  plasm. 

In  acquired  conditions  the  stoppage  is  not  so  abrupt  as  a  rule. 
For  example,  with  the  deterioration  that  goes  along  with  epilepsy  in 
childhood,  the  tests  show  much  greater  irregularity,  a  much  more 
uneven  development,  and  this  might  be  expected  of  any  condition  in 
which  deterioration  was  going  on.  In  numerous  deteriorating  con- 
ditions such  results  are  found  and  therefore  it  can  be  seen  that  one  is 
dealing  not  only  with  a  stoppage  of  development,  but  with  a  disease 
process  which,  while  it  is  in  operation,  is  producing  symptoms  of 
its  own. 

The  most  useful  classification  of  the  feeble-minded  is  a  clinical  one. 


762  IDIOCY,   IMBECILITY,   AND  DEFECT  GROUPS 

It  would  be  quite  as  absurd  to  enter  into  a  discussion  of  the  general 
psychology  or  the  general  pathology  of  defectives  as  it  would  be  of  the 
so-called  insane,  and  therefore  in  this  chapter  the  different  well-defined 
forms  will  be  briefly  and  separately  described. 

Clinical  Varieties. — Amaurotic  Family  Types  (Tay-Sachs  Disease). — 
This  is  a  disease  which  generally  affects  more  than  one  child  in  the 
family  and  appears  to  be  confined  in  its  incidence  to  the  Jewish  race. 
Its  etiology  is  unknown.  Its  pathology  is  in  general  a  degeneration 
of  certain  elements  of  the  brain,  more  particularly  the  cortical  elements 
and  pyramidal  tracts,  including  particularly  the  optic  nerves.  It  has 
been  described  in  three  stages: 


Fig.  317. — Adenoma  sebaceum.  The  skin  affection  associated  with  tuberous  sclerosis 
of  the  brain,  a  profound  degree  of  mental  defect  and  various  tumors  of  the  viscera, 
especially  the  kidneys.     (Courtesy  of  Dr.  Martin  W.  Barr.) 

First  Stage.—The  infant  is  usually  all  right  at  birth  and  the  disease 
does  not  make  itself  manifest  for  some  few  months  thereafter,  usually 
at  about  the  fourth  month.  At  this  time  the  first  symptoms  observed 
are  some  weakness  in  the  neck  muscles  and  indications  of  dimness  of 
vision.  If  the  fundus  is  examined  during  the  fourth  or  fifth  month 
there  will  be  found  a  whitish-gray  symmetrical  patch,  oval  in  shape, 
with  a  horizontal  axis  occupying  the  macula  lutea.  In  the  center  of 
this  patch  is  seen  the  fovea  centralis  which  appears  as  a  dark  cherry- 
red  spot.  Optic  atrophy  follows,  and  later  total  amaurosis.  Second 
stage:  In  this  stage  the  weakness  of  the  neck  muscles  is  more  marked 
and  the  head  falls  backward  if  unsupported,  and  while  lying  on  the 
back  the  infant  is  unable  to  turn  over  to  either  side.     The  hand 


CLINICAL  VARIETIES  763 

grasp  is  noted  to  be  feeble,  objects  are  dropped  and  the  infant  is 
generally  apathetic.  The  vision  is  materially  reduced  in  this  stage, 
but  the  senses  of  taste  and  hearing  are  preserved,  the  sense  of  hearing 
appearing  to  be  unusually  acute.  Third  stage:  In  this  stage  the 
affected  muscles  are  atrophied,  and  later  the  atrophy  extends  to  all  of 
the  muscles  of  the  body,  emaciation  becomes  marked,  the  reflexes 
exaggerated,  and  late  in  the  course  of  the  disease  the  extremities  become 
rigid,  and  there  is  retraction  of  the  head. 

Spasmodic  contractions  and  convulsions  have  been  noted.  There 
is  at  no  time  any  rise  in  temperature,  and  the  thoracic  and  abdominal 
viscera  remain  normal.     Death  usually  occurs  in  less  than  two  years. 

Sclerotic  Types. — Certain  types  of  mental  defect  are  seen  associated 
with  a  condition  of  the  brain  which  in  general  may  be  said  to  be  due 
to  an  overproduction  of  neurogliar  tissue  and  corresponding  atrophy 
and  disorientation  of  nerve  elements.  The  exact  nature  of  this 
process  is  not  definitely  known.  It  is  not  improbable  that  there  are  a 
number  of  different  conditions  comprised  in  this  general  picture. 

The  sclerosis  may  be  diffused  pretty  generally  or  it  may  be  localized 
in  patches  and  the  affected  portions  may  be  atrophic  or  they  may  be 
hypertrophic.  When  the  process  is  hypertrophic  and  involves  a  large 
portion  of  the  brain  one  finds  what  has  been  called  a  hypertrophied 
brain.  The  brain  is  much  larger  and  heavier  than  the  normal,  and 
is  much  firmer  in  consistence  after  being  hardened.  It  does  not  look 
like  a  normal  brain,  but  the  surface  has  a  cauHflower  appearance. 
The  condition  is  usually  associated  with  grave  degrees  of  mental  defect 
and  with  epilepsy.  The  localized  varieties  are  more  apt  to  be  asso- 
ciated with  convulsions  than  the  diffuse,  while  there  may  be  also  noted 
marked  tremors. 

This  disease  has  been  recently  correlated  with  other  changes  than  the 
local  cerebral  changes.  Kufs^  in  a  valuable  extensive  article,  includ- 
ing autopsy  material,  has  accumulated  the  evidence  for  a  distinct 
disease  entity  which  comprises  various  manifestations.  In  the  first 
place,  besides  these  changes  in  the  brain,  which  have  briefly  been 
referred  to,  there  is  a  distinct  cutaneous  affection  of  the  form  of 
adenoma  sebaceum,  which  affects  more  particularly  the  face  and  the 
back.  Along  with  this  condition  is  frequently  found  associated  mixed 
tumors  of  the  kidney — tumors  made  up  of  various  elements  of  which 
the  smooth  unstriped  muscle  tissue  is  the  most  prominent. 

These  three  symptoms,  then,  adenoma  sebaceum,  mixed  tumor 
of  the  kidney,  tuberous  sclerosis  of  the  brain,  are  the  triad  which 
constitute  the  most  important  evidences  of  this  disease  entity.  In 
addition  to  this  pathology  it  is  to  be  noted  first,  about  the  sclerosis 
that  it  involves  sometimes  the  cerebellum,  that  tumors  of  the  ventricles 

1  Beitrage  zur  Diagnostik  und  pathologischen  Anatomic  der  tuberosen  Hirnsklerose 
und  der  mit  ihr  Kombinierten  Nierenmischtumoren  und  Hautaffektionen  und  ilber 
den  Befund  einer  akzessorischen  Nebenniere  in  einen  Ovarium  bei  derselben.  Zeit- 
schrift  fiir  die  gesamte  Neurologie  und  Psychiatric,  Band,  xviii,  Heft  3. 


764  IDIOCY,  IMBECILITY,  AND  DEFECT  GROUPS 

are  quite  characteristic,  and  that  relatively  commonly  there  is  asso- 
ciated with  this  triad  of  symptoms  rhabdomyoma  of  the  heart.  Myoma 
of  other  organs,  such  as  the  stomach  and  the  uterus  have  also  been 
observed. 

While  on  the  mental  side,  along  with  this  condition  there  usually 
is  associated  a  marked  degree  of  mental  defect  with  epilepsy,  still 
Kirpiczniks  has  reported  a  case  occurring  in  a  man  twenty-eight 
years  old  without  mental  impairment. 

Cretinism. — This  disease  is  endemic  in  certain  parts  of  Europe, 
but  so  far  as  we  come  in  contact  with  it  in  this  country  is  sporadic. 

The  disease  is  due  to  a  defect  in  the  secretion  of  the  thj'^roid  gland. 
All  degrees  of  defect  may  be  present,  from  athyroidism  through  the 
various  degrees  of  hypothyroidism.  The  several  degrees  of  cretinism 
which  are  described  and  which  correspond  with  different  degrees  of 
defect  are  three,  namely,  the  lowest  grade,  in  which  the  defect  is 
greatest,  the  cretins,  the  middle  grades,  the  semicretins,  and  the 
higher  grades,  the  cretinoids. 

The  disease  usually  begins  quite  early  in  the  life  of  the  child,  some- 
times during  the  first  year,  although  it  may  be  delayed  for  several  years. 
The  general  symptoms  are  those,  first,  of  retarded  development;  the 
child  appears  less  bright  than  he  should  be,  walking  is  learned  slowly, 
speech  is  delayed  in  development,  the  anterior  fontanelle  is  late  in 
closing;  and  second,  the  characteristic  cretinous  appearance.  The 
body  is  dwarfed,  the  head  relatively  large,  and  the  legs  short  and 
bowed,  hands  and  feet  stumpy,  and  the  ossification  of  the  bones  is 
delayed.  The  appearance  of  the  face  is  typical,  the  nose  is  broad  and 
flattened,  the  lips  are  thick,  the  tongue  thick  and  often  protruding 
from  the  partly  opened  mouth,  the  eyes  widely  separated,  the  eyelids 
often  heavy  and  swollen,  and  the  hair  course  and  scanty.  The  skin  is 
cachectic  in  appearance  and  dry  and  thick,  resembling  the  skin  of  the 
myxedematous  patient,  the  neck  is  short,  the  abdomen  protuberant, 
with  sometimes  umbilical  hernia.  The  signs  of  puberty  are  late  in 
making  their  appearance,  there  is  often  a  failure  in  the  complete 
development  of  the  genital  organs,  and  many  of  these  patients  are 
sterile.  The  pulse  and  respiration  are  slow,  the  temperature  may  be 
subnormal,  and  the  movements  of  the  patient  are  usually  very  delib- 
erate. Mentally  the  cases  show  various  grades  of  defect,  from  the 
lowest  grades  of  idiocy  through  the  various  degrees  of  imbecility.  In 
general  cretins  are  quite  good-natured,  pliable  individuals  who  are 
easy  to  get  along  with  and  care  for. 

Diagnosis. — In  the  matter  of  diagnosis  the  principal  conditions 
which  have  to  be  differentiated  are  rickets,  achondroplasia,  and 
mongolism. 

Rickets  can  usually  be  differentiated  by  the  characteristic  beading 
of  the  ribs,  the  symmetrical  enlargement  of  the  epiphyses,  and  the 
absence  of  the  typical  signs  of  cretinism. 

Achondroplasia  should  not  be  mistaken  for  cretinism.     There  is  an 


CLINICAL   VARIETIES 


765 


imperfect  development  of  the  long  bones,  but  none  of  the  signs  of  cretin- 
ism, particularly  none  of  the  impairment  of  mental  development. 

Mongolism  is  at  times  extremely  difficult  to  differentiate.  It  is 
very  important  that  this  condition  be  not  mistaken  for  cretinism,  par- 
ticularly with  reference  to  the  matter  of  treatment,  as  treatment  is 
capable  of  modifying  the  cretin,  but  not  the  mongol.  The  following 
table  of  differential  signs  taken  from  Shuttleworth  and  Potts,i  will 
serve  to  point  out  the  detailed  characteristics  between  these  two 
conditions : 


Mongolism. 

1.  Characteristics  noticeable  from 
birth. 

2.  Skull  brachycephalic :  contour 
rounded  or  short  oval;  longitudinal  and 
transverse  diameters  nearly  correspond. 

3.  Forehead  usually  smooth. 

4.  Palpebral  fissures  "almond-shaped," 
and  more  or  less  oblique  upward  and  out- 
ward. Frequent  epicanthus.  Strabis- 
mus common.  Ciliary  blepharitis  frequent. 

5.  Cheeks  chubby,  often  florid.  Com- 
plexion mottled. 

6.  Lips  often  transversely  fissured. 
Lower  lip  may  be  pursed  up  over  upper. 

7.  Tongue  large  and  coarsely  papil- 
lated  if  not  fissured.  Tongue  frequently 
protruded  and  drawn  back. 

8.  Skin  smooth  in  infancy,  but  furfur- 
aceous  later;  not  redundant  or  "baggy." 

9.  Hair  "wiry,"  often  "mouse  color," 
but  sometimes  blonde.  Downy  growth 
common  on  forehead   and   cheeks. 

10.  Thyroid  gland  palpable  to  greater 
or  less  extent. 

11.  No  fatty  tumors  (pseudolipomata) 
in  posterior  triangle  of  neck. 

12.  Long  bones  somewhat  shorter  than 
usual,  but  slender. 

13.  Hands  broad;  thumb  and  little 
finger  short,  the  latter  often  curved  to- 
ward ring  finger.     Finger  tapers  at  ends. 

14.  Feet  large  and  flat.  Fissure  be- 
tween great  and  next  toe  often  seen. 

15.  Abdomen  often  distended;  occa- 
sional umbilical  hernia;  often  inguinal 
hernia. 

16.  Expression  more  or  less  vivacious 
and  mobile,  observant  and  imitative. 


Cretinism. 

1.  Characteristics  often  not  notice- 
able till  sixth  or  seventh  month. 

2.  Skull  dolichocephalic:  flat  at  top 
(fontanelles  close  late),  expanded  later- 
ally; broad  behind,  often  asymmetrical. 

3.  Forehead  usually  wrinkled. 

4.  Palpebral  fissures  horizontal,  but 
appear  small,  owing  to  pseudo-edema  of 
eyelids.  Strabismus  and  ciliary  bleph- 
aritis less  common. 

5.  Often  circumscribed  malar  flush; 
complexion  ashy  or  waxy. 

6.  Lower  lip  often  everted.  Mouth 
open.       Drivelling  common. 

7.  Tongue  large,  but  not  coarsely 
papillated  or  fissured.  Tip  of  tongue 
thickened,    and    constantly    protruding. 

8.  Skin  dry  and  scaly;  forms  folds 
here    and    there,    being    redundant    and 


9.  Hair  harsh,  coarse,  and  scanty. 
Usually  of  darkish  tint  (Bourneville  says 
brown) ;  scalp  often  eczematous. 

10.  Thyroid  gland  impalpable  to  most 
thorough  examination. 

11.  Fatty  tumors  (pseudolipomata) 
frequently  found  in  posterior  triangle 
of  neck,  etc. 

12.  Long  bones  shortened  and  thick- 
ened, in  some  cases  bowed. 

13.  Hands,  broad  thick,  and  stumpy, 
with  wrinkled  skin.  Fingers  square  at 
tips. 

14.  Feet  squat;  skin  redundant  about 
ankles  and  dorsum  of  foot. 

15.  Abdomen  very  bulky  and  promi- 
nent with  folds  of  skin;  umbilical  hernia 
common. 

16.  Expression  dull  and  immobile;  un- 
observant and  apathetic. 


Deficient  stature,  flattened  bridge  of  nose,  with  expanded  alse,  late  and  irregular 
dentition,  deferred  closure  of  fontanelles  and  retarded  puberty  are  similar  in  each 
variety. 

Treatment. — This  has  been  taken  up  in  Chapter  IV  on  Diseases  of 
the  Internal  Secretions,  or  Endocrinopathies. 


1  Mentally  Deficient  Children,  London,  1910. 


766 


IDIOCY,   IMBECILITY,   AND  DEFECT  GROUPS 


Mongolism. — ^The  Mongolian  or  Kalmuc  type  of  defectiveness  is 
so-called  because  of  the  resemblance  of  the  patient  to  the  Mongolian 
race,  particularly  the  slant  of  the  eyes  and  the  general  facial  expression. 

The  present  belief  with  reference  to  this  condition  is  that  it  is  con- 
genital and  not  hereditary  in  the  true  sense,  that  is,  not  dependent 
upon  a  condition  of  the  germ  plasm.  The  condition  is  believed  to  be 
the  result  of  some  abnormal  state  of  the  mother  which  may  be  produced 
by  injury  or  shock,  but  which  in  general  is  believed  to  be  the  result  of  a 


Fig.  318. — Mongolian  type,  aged  eighteen  years,  apathetic  idiot,  semimute,  speaks 
only  a  few  words,  enunciation  imperfect.  Learned  to  feed  himself  and  is  cleanly.  Dwarf- 
ish, brachycephalic,  forehead  flat  and  wrinkled  transversely.  Eyes  oblique,  photophobia, 
chronic  conjunctivitis.  Tongue  very  large,  filling  mouth  completely;  deeply  fissured, 
papillae  enlarged.  Hands  broad.  Fingers  short  and  thick.  (Courtesy  of  Dr.  Martin 
W.  Barr.) 


worn-out  reproductive  capacity,  the  mother  being  unable  to  bring  the 
child  to  complete  development  in  the  uterus.  Corresponding  with 
this  assumption  the  Mongol  is  generally  the  last  child  born  in  the 
family,  and  not  infrequently  comes  from  good  stock. 

There  are  three  prominent  physical  signs  of  this  condition  which 
are  sufficient  when  present  to  make  a  diagnosis.  They  are  the  form 
of  the  skull  and  the  peculiarities  of  the  palpebral  fissures  and  of 
the  tongue.  The  skull  is  brachycephalic,  diminished  in  its  antero- 
posterior diameter,  flattened  on  the  face  and  occiput,  but  without 


CLINICAL  VARIETIES  707 

recession  of  the  frontal  and  supraoccipital  regions,  as  in  the  micro- 
cephaHc.  The  tongue  is  large,  the  circumvallate  papillae  are  hyper- 
trophied,  and  there  are  marked  irregular  transverse  fissures.  This 
condition  of  the  tongue  is  characteristic  for  this  type,  and  is  not  found 
in  any  other  varieties.  Thompson  has  suggested  that  the  fissuration 
is  dependent  upon  two  factors :  an  extreme  vulnerability  of  the  mucous 
membrane,  and  the  habit  of  sucking  the  tongue,  commonly  present 
in  these  children.  The  hands  and  feet  are  broad,  clumsy  and  spatulate, 
while  an  incurving  of  the  little  finger  has  also  been  described  as  of 
frequent  occurrence  in  these  children.  In  addition  to  these  quite 
characteristic  symptoms  the  children  are  clumsy,  joints  loose,  the  skin 
rough,  the  abdomen  protuberant  and  there  is  a  tendency  to  chronic 
inflammatory  conditions  of  the  mucous  surfaces.  The  circulation  is 
generally  poor,  congenital  cardiac  anomalies  may  be  present,  such  as 
imperfect  closure  of  the  foramen  ovale,  vital  resistance  is  very  low, 
and  these  patients  are  quite  apt  to  die  relatively  early  in  life  from 
tuberculosis. 

Mentally  these  children  are  usually  at  a  very  low  grade  of  develop- 
ment, generally  gravitating  about  four  years  of  age.  They  may  be 
less  or  they  may  possibly  reach  the  seven-year  limit,  but  rarely  go 
beyond  it.  As  a  rule  they  are  good-natured  and  easy  to  care  for  by 
those  who  are  understanding  and  sympathetic. 

A  special  form  of  cortical  aplasia  has  been  described  as  belonging 
to  this  condition. 

Thyroid  does  no  good  in  these  conditions.  It  is  therefore  necessary 
to  carefully  separate  them  diagnostically  from  cretinism,  a  condition 
which  resembles  it  very  closely  on  casual  observation.  For  differential 
diagnosis  see  cretinism. 

Hydrocephalic  Types. — Various  degrees  of  mental  defect  may  be 
associated  with  hydrocephalus.  Hydrocephalus  may  of  course  occur 
previous  to  birth,  but  is  rarely  congenital,  as  a  child  with  any  marked 
degree  of  hydrocephalus  could  not  be  born  alive.  The  condition 
may  be  relatively  acute,  in  which  case  it  leads  rapidly  to  death,  but  it 
may  come  to  an  arrest  or  be  extremely-slow  in  progress.  In  this  latter 
group  of  cases  we  find  patients  sometimes  who  live  to  a  fairly  advanced 
age,  although  as  a  rule  this  disease  terminates  life  before  the  patient 
has  passed  middle  life  and  generally  much  younger.  The  symptoms 
in  the  slowly  progressive  cases  are  the  symptoms  of  gradual  obliteration 
of  the  mental  faculties,  and  are  undoubtedly  dependent  upon  pressure 
effects.  These  symptoms  are  in  general  loss  of  intelligence,  gradual 
loss  of  vision,  hearing,  the  function  of  language,  and  gradually  the 
sinking  into  a  semistuporous  condition,  and  death.  The  causes  of 
hydrocephalus  are  probably  numerous,  syphilis,  tuberculosis,  brain 
tumor,  and  meningitis  are  among  them. 

Microcephalic  Types. — Mental  defect  associated^  with  extreme 
smallness  of  the  head.  No  definite  rule  can  be  laid  down  as  to  what 
size  of  head  shall  be  considered  microcephalic.     Ireland  gives  the 


768 


IDIOCY,   IMBECILITY,  AND  DEFECT  GROUPS 


Fig.  319. — Hydrocephalus,  anteroposterior  diameter  9j  inches;  transverse  diameter 
7g  inches.  This  man  for  many  years  made  a  living  as  a  bell-ringer.  Previous  to  death 
he  became  profoundly  demented  and  his  head  so  heavy  that  he  could  not  lift  it  from  the 
pillow.    Autopsy  showed  the  braiii  to  be  literally  little  else  than  a  bag  of  fluid. 


Fig.  320. — On  left,  high-grade  imbecile,   aged  thirteen  years;  mentally,  six  years. 
On  right,  microcephalic  imbecile,  aged  nine  years ;  mentally,  three  years. 


CLINICAL   VARIETIES 


769 


general    rule   that   heads   below   seventeen   inches   in   circumference 
(431  miUimeters)  may  be  so  considered.     This  rule  is  not  absolute, 


Fig.  321. — Erma.     Front  view. 


Fig.  322. — Erma.     Side  view. 


Fig.  32:1 — Mary.     Front  view.  Fig.  324. — Mary.     Side  view. 

Microcephalic  twins,  aged  eight  years.      (Courtesy  of  Dr.  A.  C.  Rogers,  Faribault, 

Minnesota.) 
49 


770  IDIOCY,   IMBECILITY,   AND  DEFECT  GROUPS 

and  when  it  is  considered  that  these  small-headed  varieties  may  prob- 
ably be  the  result  of  various  etiological  factors  it  will  be  appreciated 
that  the  term  microcephalic  had  better  be  used  purely  as  a  descriptive 
term  rather  than  as  a  term  to  apply  to  a  definite  class  of  defectives 
even  though  that  class  be  considered  solely  from  a  morphological 
standpoint. 

It  is  probable  that  two  sets  of  causes  may  produce  the  extremely 
small  skulls  which  are  found  in  the  microcephalic  types.  The  old 
theory  that  the  condition  was  due  to  premature  synostosis  has  long 
since  been  discarded  as  has  also  the  operation  of  craniectomy  based 
upon  that  theory. 

The  characteristic  condition  of  the  microcephalic  brain  is  its  extreme 
smallness,  more  particularly  pronounced  in  the  temporosphenoidal, 
parietal  and  occipital  regions.  The  posterial  lobes  of  the  cerebrum 
do  not  cover  the  cerebellum.  The  convolutions  of  the  cerebrum  are 
more  simple  in  pattern  than  in  the  normal  brain,  and  in  addition  there 


Fig.  325. — Paralytic  type.     Athetoid  movements  of  hands  and  arms. 

may  be  localized  ageneses  with  resulting  microgyria.  There  may  also 
be  associated  morbid  processes  such  as  encephalitis.  The  hypoplasia 
usually  also  involves  the  spinal  cord. 

The  general  appearance  of  these  patients  is  quite  characteristic. 
The  confirmation  of  the  skull  is  "sugar  loaf,"  or  as  it  is  technically 
termed  "oxycephalic."  This  is  characterized  by  a  rapidly  receding 
forehead  with  a  flat  occiput.  Along  with  the  receding  forehead  there  is 
usually  also  a  receding  chin  which  gives  a  pointed  aspect  to  the  face, 
which  associated  with  a  small  stature  gives  a  quite  characteristic 
general  appearance  to  these  patients  and  has  lead  to  their  being 
characterized  as  "bird-like"  in  appearance. 

The  mental  development  of  these  patients  varies  between  wide 
limits,  although  the  tendency  is  for  them  to  belong  to  the  lower  grades 
of  defect.  They  are  usually  well  disposed,  good-natured,  affectionate, 
and  not  difficult  to  care  for.  They  generally  do  not  live  to  advanced 
years,  but  die  at  rather  an  early  age. 


CLINICAL  VARIETIES 


771 


Paralytic  Types. — ^There  are  a  large  variety  of  cases  in  this  group. 
The  paralyses  may  involve  any  portion  of  the  body  or  be  of  almost 
any  extent.  Monoplegias  and  diplegias  are  common,  but  localized 
palsies  and  hemiplegias  are  also  not  infrequent.  They  depend  either 
upon  lack  of  development  of  certain  portions  of  the  brain  or  more 
frequently  upon  injuries  and  sometimes  new  growths.  Hemorrhage 
is  the  most  frequent  injury  that  produces  the  various  palsies.  This 
may  occur  as  the  result  of  prolonged  labor  or  injury  by  forceps  delivery, 
or  may  be  the  result  of  injury  during  the 
early  months  of  life.  The  amount  and 
the  character  of  the  mental  defect  varies 
within  wide  limits  and  no  description 
which  would  apply  to  this  whole  class 
"would  be  possible,  principally  because  the 
class  is  riot  a  homogeneous  one. 

Porencephalus. — ^Within  this  group  of 
paralytic  types  one  of  the  conditions 
which  is  found  and  which  has  been  fre- 
quently described  is  porencephalus.  This 
condition  is  consequent  upon  gross  cere- 
bral lesions  such  as  a  lack  of  cerebral 
substance  resulting  in  a  cyst  connected 
with  the  ventricle — true  porencephalus, 
or  due  to  cysts  not  connecting  with  the 
ventricles  and  resulting  from  softening, 
hemorrhage,  or  inflammation — false  por- 
encephalus. 

Traumatic  Types. — ^Arrest  of  mental 
development  may  occur  as  the  result  of 
an  injury  to  the  brain  during  the  de- 
velopmental period.  The  most  frequent 
type  of  injury  producing  this  result  is 
prolonged  labor  with  instrumental  de- 
livery. Where  the  injury  produces  a 
lesion  in  the  motor  pathway  with  a  re- 
sulting paralysis  the  patient  is  generally 
included  within  the  paralytic  group. 

Epileptic  Types. — Epilepsy  occurring 
early  in  life    is  one    of  the  causes    of 

lack  of  mental  development.  Just  how  this  result  is  brought  about 
by  the  epilepsy  is  not  altogether  clear.  In  part  it  is  due  to  the 
fact  that  the  child  has  to  be  differently  treated  from  normal  children, 
is  often  deprived  of  the  same  educational  advantages  because  of  its 
disease,  but  apart  from  this  there  seems  to  be  a  direct  relation  between 
the  lack  of  development  and  mental  deterioration,  and  the  epilepsy 
itself.  This  is  probably  dependent  upon  the  fact  that  the  epilepsy 
is  a  disease  which  strikes  very  deep,  close  to  the  foundation  of  the 


Fig.  326. — Hemiplegic,  aged 
eleven  years.  (Courtesy  of  Dr. 
A.  C.  Rogers,  Faribault,  Min- 
nesota.) 


772  IDIOCY,  IMBECILITY,  AND  DEFECT  GROUPS 

neurological  mechanisms.  Its  manifestations  are  evidently  in  part, 
at  least,  dependent  upon  disturbances  at  the  physico-chemical  level. 
Disturbances  which  are  as  fundamental  as  this  necessarily  are  diffi- 
cult to  deal  with  and  also  necessarily  impair  the  superposed  levels. 
(See  Chapter  on  Epilepsy.) 

Epileptic  attacks  are  found  in  many  of  the  other  forms  of  defect, 
more  particularly  in  those  defects  associated  with  gross  cerebral 
lesions  such  as  are  found  in  the  paralytic  and  traumatic  types.  It  has 
generally  been  assumed  that  the  localized  lesion  was  the  cause  of  the 
epileptic  manifestations.  While  this  may  be  so  in  certain  cases,  still 
in  a  general  way  it  must  be  borne  in  mind  that  epilepsy  is  presumably 


Fig.  327. — Sensorial  type,  imbecile  by  deprivation,  middle  grade.  Male,  aged  about 
thirty  years,  deaf-mute.  Taught  himself  to  talk  and  to  read  and  write  in  a  fashion. 
Often  cruel  to  children  but  devoted  to  animals.  Appropriates  things  not  his  own. 
Very  keen  and  observant.     (Courtesy  of  Dr.  Martin  W.  Barr.) 

an  hereditary  disease  dependent  upon  a  defect  in  the  germ  plasm  and 
that  perhaps  only  those  children  develop  convulsions  as  a  result  of 
injury  or  localized  cerebral  lesions  in  whom  hereditary  conditions  are 
favorable  for  the  outcrop  of  epilepsy.  At  least  the  hereditary  factor 
should  not  be  neglected  in  the  study  of  the  patient  simply  because  a 
localized  lesion  has  been  found.  Perhaps  the  localized  lesion  could 
not  have  produced  such  a  result  without  the  hereditary  factor — the 
spasmophilic  tendency. 

The  epileptic  type  of  defective,  besides  the  symptoms  of  his  defect, 
characteristically  manifests  the  symptoms  of  the  epileptic  character 
and  is  therefore  quite  a  difficult  problem  with  which  to  deal. 


CLINICAL  VARIETIES 


773 


Inflammatory  Types. — This  group  includes  those  conditions  resulting 
from  inflammation  of  the  meninges  and  of  the  brain,  found  most 
frequently  as  a  result  of  acute  infectious  fevers  such  as  pneumonia, 
typhoid,  and  the  exanthemata.  Local  areas  of  meningitis  or  meningo- 
encephalitis are  not  infrequent  in  connection  with  the  specific  fevers, 
and  when  extensive  or  severe  and  occurring  early  in  life  produce  an 
arrest  of  development,  to  a  certain  degree,  of  the  mental  faculties 
together  not  infrequently  with  epilepsy.  Striimpell's  polioencephalitis 
superior  belongs  in  this  group. 

Sensorial  Types. — The  defect  in  this  group  is  the  result  of  deprivation. 
Here  there  is  no  defect  of  the  germ  plasm  or  no  defect  in  the  structure 
of  the  brain,  but  owing  to  injury  or  disease  which  has  destroyed 


Fig.  328. — Sensorial  type,  imbecile  by  deprivation,  middle  grade.     Boy,  aged  ten 
years,  deaf-mute,  no  ears.     (Courtesy  of  Dr.  Martin  W.  Barr.) 

the  patient's  vision  or  hearing  or  both  the  child  is  cut  off  from  com- 
munication with  the  outside  world  to  such  an  extent  that  mental 
development  is  imjDaired  thereby.  Theoretically  these  patients  are 
of  course  educable,  but  it  is  only  very  rarely  that  one  is  found  with  the 
capacity,  the  patience,  and  the  ingenuity  to  develop  such  a  child  by 
educational  methods.  The  cases  of  Helen  Keller  and  Laura  Bridgman 
stand  out  as  illustrative  of  what  can  be  done. 

Syphilitic  Types.— Syphilitic  types  of  defect  have  always  been 
known  in  that  group  of  cases  presenting  distinct  evidences  of  hereditary 
syphilis,  particularly  the  Hutchinson  teeth,  linear  scars  about  the 
mouth  and  nose,  and  keratitis.  Until,  however,  the  discovery  of  the 
Treponema  pallidum  and  the  elaboration  of  the  Wassermann  reaction 
there  was  no  comprehension  of  the  frequency  with  which  syphilis 


774  IDIOCY,  IMBECILITY,  AND  DEFECT  GROUPS 

entered  into  the  etiology  of  the  various  types  of  mental  defect.  One 
of  the  best-known  authors,  who  just  antedated  this  period,  says  that 
the  number  of  cases  of  defectives  due  to  syphilis  is  quite  insignificant, 
probably  not  more  than  one  or  two  per  cent.,  and  comments  upon 
this  in  the  face  of  the  frequency  of  syphilitic  disease  in  the  fully 
developed  brain  and  the  frequency  of  so-called  inherited  syphilis. 

There  is  one  distinct  type  of  disease  which  only  recently  has  come 
to  be  recognized  with  any  degree  of  accuracy  which  used  to  be  classed 
among  the  defective  states  and  which  is  due  to  syphilis,  namely, 
juvenile  general  paresis.  It  is  probable  that  still  in  many  places  this 
disease  is  not  recognized,  but  is  put  down  probably  as  some  form  of 
progressive  defect.     The  number  of  juvenile  paretics  is  not  very  large. 

The  application  of  the  Wassermann  test  to  the  defectives  as  a  class 
shows  that  not  far  from  20  per  cent,  of  the  patients  taken  indiscrim- 
inately show  a  positive  Wassermann  reaction.  Of  course  it  is  quite 
another  question  as  to  just  what  the  relationship  is  in  these  patients 
between  the  syphilis  and  the  mental  defect.  In  one  case  it  may  be 
that  the  syphilis  has  produced  vascular  disease  and  the  defect  is  due  to 
a  vascular  lesion.  In  another  case  it  is  the  typical  effect  of  a  general 
paresis,  or  perhaps  a  meningo-encephalitis,  and  still  further  probably 
syphilis  has  a  decidedly  deleterious  effect  upon  development  in  ways 
that  we  do  not  at  present  understand,  so  that  it  may  well  be  that 
certain  of  the  types  of  defect  which  do  not  present  characteristic 
syphilitic  lesions  may  be  due  to  subtle  nutritional  changes,  which,  in 
the  last  analysis,  have  their  origin  in  syphilis.  The  great  part  that  this 
disease  plays  in  this  class  of  cases  is  yearly  becoming  more  and  more 
evident. 

Idiot-savants.  —  These  are  rare  cases,  who,  although  often  deeply 
defective,  still  have  some  special  ability  wonderfully  developed.  It 
may  be  music,  calculation,  or  memory  for  some  special  class  of  facts,  etc. 

The  calculators  can  name  the  answer  to  mathematical  problems 
almost  instantly;  the  musical  prodigies  often  play  well  and  may  even 
improvise;  one  patient  under  the  observation  of  one  of  the  authors 
would  instantly  name  the  day  of  the  week  for  any  date  for  years 
back.  Many  of  these  patients  have  a  capacity  for  mimicry  and 
buffoonery,  and  from  this  class  undoubtedly  were  recruited  in  the 
old  days  many  of  the  court  fools. 

The  psychology  of  these  individuals  is  not  understood  and  they 
themselves  are  quite  unable  to  give  any  explanation  of  their  special 
abilities.  Their  abilities,  however,  are  really  not  so  great  as  they 
appear.  They  appear  exaggerated  because  they  stand  out  upon  a 
background  of  pronounced  defect,  also  because  they  are  unusual 
in  the  sense  of  not  being  the  common  possession  of  mankind.  The 
calculators,  for  example,  do  very  wonderful  calculations  in  the  way  of 
adding  up  long  series  of  figures  very  rapidly.  There  are,  however, 
well-known  devices  for  increasing  the  rapidity  of  the  ordinary  old- 
fashioned  method  of  adding,  and  many  of  our  experts  at  figures  today 


CLINICAL  VARIETIES 


775 


could  compete  with  some  degree  of  success  with  these  calculators. 
The  patient,  for  example,  who  could  name  the  day  of  the  week  for 
years  back  upon  merely  being  given  the  date,  and  do  it  instantly, 
spent  most  of  his  time  in  studying  calendars.  It  would  not  be  surpris- 
ing if  almost  anyone  could  accomplish  such  a  feat  if  he  spent  any 
such  commensurate  degree  of  effort  upon  it. 

Other  descriptive  terms  are  used  to  describe  certain  types  of  defec- 
tives, for  instance  in  addition  to  the  so-called  Mongol  or  Kalmuc  types 
there  are  described  American  Indian  and  Negroid  types  because  of  the 
obvious  resemblances.  Then  again,  for  purposes  of  practical  classifica- 
tion the  lower  grade  of  defectives  are  spoken  of  as  either  apathetic 
or  excitable,  and  certain  of  these  latter  who  keep  up  certain  definite 
and  characteristic  movements  almost  continuously  are  described  as 


Fig.  329.  —  American  Indian 
type.  (Courtesy  of  Dr.  Martin 
W.  Barr.) 


Fig. 


330. — Negroid  type.     (Courtesy  of 
Dr.  Martin  W.  Barr.) 


rhythmic  idiots.  Other  motor  disturbances  of  course  also  occur.  Aside 
from  paralysis  and  epilepsy,  probably  one  of  the  most  common  is 
athetosis. 

Mild  Grades  of  Defect. — A  systematic  examination  of  large  numbers 
of  children  has  disclosed  the  fact  that  a  great  number  of  the  disorders 
of  conduct  and  types  of  inefficiency  which  are  manifested  among  them 
are  dependent  upon  some  degree  of  feeble-mindedness.  Systematic 
examinations  of  school  children,  for  example,  have  resulted  in  showing 
a  not  inconsiderable  percentage  of  the  general  school  population  so  far 
behind  in  their  mental  development  as  only  to  be  described  by  a  term 
indicating  an  inherent  defect.  These  defects  range  all  the  way  from 
well-marked  imbecility  among  the  younger  children  up  through  the 
grades  of  the  so-called  backward  children.     For  the  most  part,  the 


776 


IDIOCY,  IMBECILITY,  AND  DEFECT  GROUPS 


different  grades  of  the  moron,  of  the  feeble-minded,  and  the  lighter 
measuring  rod  which  has  been  used  for  determining  these  conditions 
has  been  the  Binet-Simon  scale  of  intelligence  tests,  and  the  treatment 
which  has  been  applied  has  been  the  segregation  of  these  defective 
children  from  the  general  school  population  into  classes  and  sometimes 
whole  schools  devoted  particularly  to  them,  thereby  gaining  both  the 
advantage  of  the  application  of  special  educational  efforts  to  these 
children  and  the  relief  of  the  normal  child  from  the  drag  back  to  which 
he  was  subjected  by  having  the  defective  in  the  same  class  with  him. 

Many  of  the  mental  defects,  it  must  be  borne  in  mind,  are  only 
relative  affairs  and  are  dependent  upon  general  conditions  of  ill  health, 


Fig.  331. — In  center  a  moron,  aged  twenty-four  years;  mentally,  ten  years.  At  right, 
moron,  aged  eleven  years;  mentally,  eight  years.  At  left,  imbecile,  aged  nine  years; 
mentally,  six  years. 

such,  for  example,  as  are  due  to  adenoid  vegetations  in  the  posterior 
pharynx.  Under  such  conditions  of  ill  health  development  is  impaired 
and  does  not  proceed  at  a  normal  rate.  With  anemia,  impaired 
digestion,  and  infected  tonsils,  which  produce  a  constant  toxemia, 
the  child  cannot  be  expected  to  proceed  in  his  development  with  normal 
rapidity.  In  addition  to  such  conditions  as  this  it  is  found  that  the 
defect  is  often  due  to  high  grades  of  myopia  which  make  it  impossible 
for  the  child  to  learn,  because  he  cannot  see  to  read  or  even  see  the 
blackboard.  In  the  same  way  deafness  and  other  quite  gross  lesions 
have  been  found  to  account  for  many  of  these  conditions.  It  should  be 
remembered,  too,  that  the  child  may  have  a  neurosis  or  a  psychoneurosis, 


CLINICAL   VARIETIES  777 

and  that  these  conditions  may  produce  conduct  disorders  which  show 
a  moral  obhquity  which,  if  they  are  not  carefully  investigated,  may 
too  easily  be  classified  as  due  to  defective  conditions.  Such  psychic 
states  are  susceptible  of  psycho-analysis,  and  often  of  cure. 

Psychopathic  Constitution. — There  are  many  anomalies  of  char- 
acter which,  because  normal  or  usual  to  the  indi^•idual,  cannot  be  said 
to  properly  constitute  a  psychosis,  but  because  they  lead  to  a  rather 
inefficient  type  of  adjustment  of  the  individual  to  his  environment, 
and  because  persons  exliibiting  these  peculiarities  often  become 
actively  disordered,  may  be  considered  as  borderland  conditions. 

Something  of  the  hysteric  and  epileptic  characters  has  already 
been  discussed.  The  general  type  of  inefficiency  of  the  neurasthenic 
and  the  psychasthenic  character  has  been  described,  and  attention 
has  been  called  to  the  unresistiTC  and  the  post-trawnatic  types  with 
their  intolerance  of  alcohol  and  fever.  There  also  may  be  said  to  exist 
a  manic-depressive  t^'pe  and  lately  attention  has  been  directed  to  the 
"shut-in'  type  as  the  type  par  excellence  found  in  those  cases  which 
develop  dementia  precox. 

Besides  these  there  are  the  "cranks,"  who  with  some  pet  scheme, 
closely  approach  the  paranoiac  type  and  that  host  of  ill-balanced, 
eccentric  individuals  who  may  be  superficially  brilliant  but  lack  con- 
tinuity of  purpose  and  capacity  for  the  continuous  expenditure  of 
effort  in  any  one  direction.  Their  life,  to  use  the  well-chosen  words 
of  Regis,  is  one  "long  contradiction  between  the  apparent  wealth  of 
means  and  poverty  of  results." 

There  are  also  the  pathological  liar,  or  pseudologia  phantastica,  and 
certain  types  of  swindlers. 

Constitutional  anomalies  of  mood  are  seen,  those  who  are  always 
depressed — psychopathic  depression — and  the  opposite  state — psycho- 
pathic exaltation.  Others  never  seem  to  be  quite  able  to  successfully 
cope  with  conditions;  they  are  the  failures  of  life,  the  cases  of  constitu- 
tional inferiority. 

Conditions  of  psychogenic  depression  occur  quite  often  in  psycho- 
pathic individuals.  ]Many  weak  characters  who  are  led  into  crime 
develop  a  symptomatic  depression  when  caught  and  sentenced.  More 
pronounced  defects  of  character  are  seen  in  the  criminal  classes,  many 
of  whom  lack  the  ordinary  moral  inhibitions  and  are  properly  morcd 
imbeciles. 

There  are  many  psychogenic  states  that  occur  in  psychopathic 
individuals — deviates.  The  so-called  prison  psychoses  are  types  and 
come  about  as  the  patient's  reaction  to  the  difficulties  in  which  he 
finds  himself.  They  may  be  hysterical,  catatonic,  paranoid,  accord- 
ing to  the  type  of  individual.  They  clear  up  when  the  stress  is  removed 
— pardon,  expiration  of  sentence,  commutation  of  sentence,  etc. 


778  IDIOCY,  IMBECILITY,  AND  DEFECT  GROUPS 


ANOMALIES  OF  THE  SEXUAL  INSTINCT. 

Quantitative  Anomalies.— These  are  frigidity  or  lack  of  desire  for 
sexual  congress — sexual  anesthesia — or  eroticism — sexual  hyperesthesia. 

Qualitative  Anomalies. — These  are  inmrsions  and  'perversions. 
Inversion  consists  of  a  lack  of  harmony  between  the  physical  and  the 
psychical  sex  and  leads  to  homosexuality  or  desire  for  persons  of  the 
same  sex.  Various  physical  anomalies  are  often  found  in  these 
persons.  For  example,  the  general  conformation  of  the  body,  pilosity, 
etc.,  may  indicate  one  sex,  while  the  genitalia  are  of  the  other. 

Sex  inheritance  is  alternative.  That  is,  both  male  and  female  char- 
acters are  present  in  the  germ  and  only  one  normally  develops.  Some- 
times there  seems  to  be  an  uncertainty  as  to  which  will  develop  and 
the  result  is  a  certain  mixture  which  may  take  place  either  in  the 
bodily  or  psychic  sphere  alone  or  in  both. 

The  perversions  are  many  and  include  the  various  anomalous  means 
of  gratifying  the  sexual  appetite. 

With  respect  both  to  inversion  and  perversion  it  must  be  remem- 
bered that  in  the  young  child  the  sexual  instinct  has  not  developed  and 
later  as  it  develops  and  comes  into  prominence  it  differentiates  and 
tends  to  specialize  by  centering  its  aims  in  a  special  direction,  i.  e., 
toward  the  opposite  sex  and  normal  coitus.  The  child,  before  this 
takes  place  is,  to  use  a  term  of  Freud's,  polymorphous-perverse.  He 
may  be  developed  in  any  direction  by  appropriate  influences  or  he  may 
stay  in  the  undeveloped,  infantile  stage. 

The  most  important  of  the  perversions  are: 

Masturbation. — Masturbation  is  very  frequent  among  psychopaths 
and  very  often  a  result  rather  than  a  cause  of  mental  anomalies,  though 
undoubtedly  an  important  factor  in  some  cases  of  acute  psychosis. 
A  transient  period  of  onanism  in  infancy  is  probably  normal  and 
serves  to  focalize  the  sexual  sensations  on  the  normal  erogenous  zones. 

Active  Algolagnia  (Sadism) .—The  gratification  of  the  sexual  feeling 
by  the  infliction  or  sight  of  pain — real  or  simulated.  In  the  latter  case 
the  sadism  is  symbolic.  As  the  male  is  normally  the  more  active  and 
aggressive  in  the  sexual  relation,  as  might  be  expected,  this  anomaly 
is  more  frequently  found  in  men. 

Passive  Algolagnia  (Masochism). — The  gratification  of  the  sexual 
feeling  by  suffering  pain— real  or  simulated.  In  the  latter  case  it  is 
symbolic.  The  female,  being  the  more  passive  of  the  two  sexes  in  the 
sexual  relation,  so  an  exaggeration  of  this  passivity  is  more  frequently 
found  among  women. 

Homosexuality. — Sexual  desire  for  the  same  sex. 

Narcissism. — A  form  of  auto-erotic  sexuality  in  which  a  person  is  in 
love  with  himself — his  own  body. 

Fetichism.— Sexual  excitement  and  gratification  by  the  sight,  con- 
tact or  possession  of  some  object  or  part  of  the  body.     The  object 


ANOMALIES  OF  THE  SEXUAL  INSTINCT  779 

is  usually  some  wearing  apparel,  such  as  shoes,  handkerchief,  petticoat, 
or  a  part  of  the  body  other  than  the  sexual  organs. 

Bestiality. — Sexual  relation  with  animals. 

Exhibitionism. — Sexual  gratification  by  exposing  the  genital  organs. 

Necrophilia. — The  desire  to  have  sexual  congress  with  a  dead  body. 

Most  of  these  conditions  stand  for  what  was  normal  at  a  certain 
stage  in  development  but  should  have  been  left  behind  in  the  progress ; 
or  else  they  are  the  result  of  aberrant  development  from  these  lower 
points  when  there  has  been  a  stagnation  of  the  developmental  process 
and  so  are  included  in  this  chapter.  The  higher  psychic  ramifications 
are  fully  discussed  in  the  chapters  on  the  neuroses,  psychoneuroses, 
epilepsy,  and  certain  psychoses,  notably  dementia  precox  and  manic- 
depressive  psychosis,  in  all  of  which  disturbances  of  psychosexual 
development  are  present. 


INDEX. 


Abderhalden  reaction,  96 
Abducens,  197 

nerve,  tests  of,  31 
palsy,  202 
Abscess  of  brain,  course,  463 
diagnosis,  464 
etiology,  462 
forms,  463 
history,  462 
otitic,  464 
prognosis,  465 
rhinogenic,  464 
symptoms,  462 
traumatic,  463 
treatment,  465 
cerebellar,  419 
Acoustic  nerve,  distribution  of,  223 
paths,  223 

pathways,  central,  224 
Acromegaly,  161 
Acroparesthesia,  277 
Actinomycosis  of  brain,  495 
Addison's  disease,  167 
Adenoids,  26 
Adenomata  of  brain,  494 
Adiadokokinesis,  55,  407 
Adiposis  dolorosa,  166 
AgenitaUsm,  167 
Agraphias,  244 
Alcoholic  epilepsy,  727 
hallucinosis,  725 
pseudoparanoia,  726 
pseudoparesis,  726 
Alcoholism,  chronic,  721 
delirium  tremens,  722 
dream  states,  727 
drunkenness,  pathological,  721 
psychology  of,  719 
Alexias,  244 
Algolagnia,  778 
Allo-erotism,  672 

Alternating  insanity.    See  Manic-depres- 
sive psychosis. 
Alzheimer's  disease,  751 
Amentia,  716 
Amnesic  aphasia,  224 
Amyotonia  congenita,  334 
Amyotrophic  lateral  sclerosis,  amyotro- 
phic type,  340 
course  and  dura- 
tion, 340 


Amyotrophic  lateral  sclerosis,  amyotro- 
phic type,  diag- 
nosis, 340 
etiology,  337 
history,  337 
labio-glosso-laryn- 

geal  type,  340 
pathology,  337 
spastic  type,  340 
spinal  type,  ordi- 
nary, 339 
symptoms,  338 
treatment,  340 
Anamnesis,  17 
Anarthria,  41 
Angiomata  of  brain,  494 
Angioneurotic  edema,  123 

forms,  transition,  127 
heredity  in,  124 
pathogenesis,  127 
symptoms,  125 
treatment,  128 
Anxietas  prsesenilis,  745 
Anxiety,  614 

distm-bances,  gastro-intestinal  func- 
tions in,  617 
free  floating,  613 

neurosis,  anxious  expectation,  613 
cardiac  disturbances,  615 
etiology  and  occm-rence,  618 
irritability,  general,  613 
phobias  in,  616 

respiratory  disturbances  in,  615 
symptoms,  613 
vertigo  in,  615 
Aphasia,  240,  487 
amnesic,  224 
conduction,  243 
motor,  242 

cortical,  243 
subcortical,  243 
transcortical,  243 
partial,  242 
regions  of,  240 
sensory,  242 

cortical,  243 
subcortical,  243 
transcortical,  243 
total,  242 
types,  243 
Aplasias,    combined,  of  cerebellum  and 

brain,  413 
Apoplectic  attack,  471 


782 


INDEX 


Apoplexy,  cerebral,  466,  470 
coma  in,  472 
complexes,  irritative  in,  473 
confusion  in,  473 
diagnosis,  487 

differential,  489 
Jacksonian  attacks  in,  473 
meningeal,  441 
middle      cerebral     syndromes      in, 

474 
prognosis,  490 
psychoses  of,  732 
temperature  in,  472 
treatment,  491 
Apraxia,  64,  484 
eyeball,  209 
facial  musculature,  218 
ideomotor,  487 
intrapsychic,  485 
motor,  485 
sensory,  485 
tests  for,  55 
Arachnoid,  diseases  of,  446 
Aran-Duchenne  type  muscular  atrophy, 

320,  321 
Argyll-Robertson  pupil,  85,  201,  520 

mechanism  of,  34 
Arteriosclerosis,  cerebral,  468 
diagnosis,  756 
paresis  and,  756 
pathology,  755 
symptoms,  469,  753 
treatment,  756 
perivascular  gliosis,  753 
senile  cortical  devastation,  753 
subcortical  encephalitis,  752 
Ai-teriosclerotic  brain  atrophy,  752 

psychoses,  752 
Artlu-itic  disturbances  in  ancestry,  24 
Arthritis,  27 

Associated  movements,  55 
Association,  free,  76,  626 
Astereognosis,  64 
Asthenia,  410 
Asynergia,  38 
Ataxia,  tests  for,  55 
Ataxic  paraplegia,  354 
Athetoid  movements,  55 
Attainment,  594 

Auditory     disturbances,     summary    of, 
225 
nerve,  anatomy  of,  222 
diseases  of,  222,  223 
tests  of  cochlear  portion,  37 
vestibular  portion,  38 
Autistic  thinking,  685 
Auto-erotic  stage,  597 
Auto-erotism,  672 

Autonomic  division  of  vegetative  nervous 
system,  82 
nervous  system,  79 
and  sympathetic  nervous  systems, 
comparison  of  antagonistic  actions 
of,  84 


B 


Babinski  asynergic  tests,  38 

reflex,  52 
Baby,  mental  life  of,  594 
Barany  tests,  31,  32,  39,  229 
Benedict  syndrome,  197,  201,  420 

contralateral,  386 
Bestiality,  779 
Binet-Simon  tests,  760 
Bladder  mechanisms,  95 
Bloodvessels,  innervation  of,  100 
Bonnier's  syndrome,  230 
Bony  sensibility,  58 
tests  of,  63 
Bourdon  test,  72 
Bradylalia,  41 
Brain,  anemia,  468 

arteriosclerosis  of,  468 

diseases  of,  458 

hyperemia,  468 

stem,  diseases  of,  385 

vascular  disturbances  of,  466 
instability  of,  468 

vessels  of,  466,  467 
Brown-Sequard  syndromes,  344,  346,  347 
Bulbar  palsies,  chronic  progressive,  323 


Cachexia  thyreopriva,  134 
Carbon  bisulphide  intoxication,  285 

monoxide  intoxication,  285 
Carcinomata  of  brain,  494 
Cardiac  mechanism,  98,  99,  100 
Cataleptic  rigidity  in  cerebellar  defect, 

39 
Central  nystagmus,  203,  209 
Cephalogyric  fibers,  198 
Cerebellar  abscess,  419 

aplasias  and  ageneses,  411 
asynergia,  410 
ataxia,  405 

conditions,  treatment  of,  422 
cysts,  416 

disease,  symptoms  of,  405 
dysmetria,  407 
functions,  399 
gait,  406 
hemorrhage,  415 
hypotonus,  409 

and  labyrinthine  disturbances,  dif- 
ferential diagnosis  of,  233 
peduncles,  400 

inferior,  paths,  400 
syndrome,  410 
middle,  paths,  401 
syndrome,  411 
posterior,  fiber  tracts,  402 
superior,  paths,  401 
syndrome,  411 
and  pontine  tracts,  descending,  404 
syndromes,  399 


INDEX 


783 


Cerebellar  syndromes,  chief,  410 
tracts,  402 

of  spinal  cord,  ascending,  403 
tumors,  416  , 

posterior  fossa  complications  of, 
420 
vertigoes,  230 
Cerebellopontine  angle  tumors,  421 
Cerebellum  connections,  spinal,  227 
with  forebrain,  228 
and  cord  atrophies  and  aplasias,  413 
primary  parenchymatous  degenera- 
tion of,  415 
Cerebral  arteriosclerosis,  468 

symptoms  of,  469 
Cerebrospinal  fluid,  Alzheimer's  method 
of  examination  of,  516 
chemical     examination     of,    in 
paresis,  541 
in  syphilis,    514,  515, 
517 
Cervical  rib,  290,  300 
Chaddock  reflex,  54 
Character,  types  of,  777 
Charcot-Marie-Tooth  type  of  muscular 

atrophy,  325 
Chiasm,  diseases  at  or  about  the,  193 
Chloromata  of  brain,  494 
Cholesteomata  of  brain,  494 
Chordomata  of  brain,  494 
Chorea,  acute,  psychoses  of,  735 
chronic,  psychoses  of,  736 
chronica,  433 

degenerans  of  Brissaud,  433 
electrica  of  Bergeron  and  Henoch, 
433 
of  Dubini,  433 
epileptica,  434 
Huntington's,  433,  437 
course,  440 
etiology,  438 
pathology,  440 
symptoms,  438 
mental,  439 
motor,  438 
therapy,  440 
insaniens,  718,  736 
minor,  433,  434 

cerebellar  signs,  436 

diagnosis,  437 

etiology,  436 

pathogenesis,  436 

pyramidal   tract   disturbances, 

435 
symptoms,  434 
treatment,  437    . 
postapoplectica,  434 
posthemiplegic,  434 
tabica,  434 
thalamica,  434 
Choreas,  423,  433 
cerebellar,  434 

of   congenital   or   infantile   palsies, 
434 


Choreas  of  general  paresis,  434 
of  psychoses,  434 
superior  cerebellar  peduncle,  434 
Choreic  movements,  55 
Circular  insanity.    See  Manic-depressive 

psychoses. 
Circumflex  nerve,  301 
Claudication,  intermittent,  252 
Cocainism,  728 
Collapse  delirium,  715 
Combined  degenerations,  353 
scleroses,  353,  354 

ataxic  paraplegia  types,  354 
senile  forms,  355 
spastic  ataxia  type,  354 
toxic  forms,  355 
Complex,  the,  72 

Compulsion  neurosis,  anxiety  in,  608 
ceremonials,  609,  610 
compromise     symptoms,     607, 

608 
compulsive  action,  608,  609,  611 

thinking,  609 
doubts  in,  609,  611 
fears  in,  608 
hypochondria  in,  608 
manias  in,  611 
mechanism  of,  605 
melancholia  in,  608 
neurasthenia  in,  608 
obsessions,  606,  607,  609 
phobias,  608,  609,  611 
repression  in,  607 
reproaches  in,  606 
secondary  defense  of  obsessions, 

609 
symptoms,  611 
Conflict,  the,  595 

Confusion,  acute  hallucinatory,  716 
Conjugate  deviation,  203 
Consanguinity,  18,  19,  20,  21,  22 
Constitution,  general,  28 
Constitutional  inferiority,  777 
Continuous  bath,  652 
Convergence,  paralysis  of,  203,  207 
Convulsive  seizures,  27 
Cortex,   disease  of,   in  lesions  of  optic 
tract,  195 
as  organ  of  local  attention,  481 
thalamus  and,  functional  relations, 
482 
Cortical  lesions,  sensory  changes  in,  478, 

479,  480 
Cranial  nerves,  29,  69,  179 
Cretinism,  133,  137,  764 
and  deaf-mutism,  142 
endemic,  140 
and  mongoHsm,  765 
sporadic,  137 
therapy  of,  142 
treatment  of,  765 
Cretinoid  degeneration,  139 
Cutaneous  nerve,  external,  302 
Cycloplegia,  209 


784 


INDEX 


Cyclothymia.  See  Manic-depressive 
psychosis. 

Cysticercus  of  brain,  495 

Cysts  of  cerebellum,  416 

Cytology  of  cerebrospinal  fluid  in  syph- 
ilis, 515 


Dalrymple  sign,  145 
Day-dreaming,  604 
Deaf-mutism,  endemic,  142 
Deafness,  223 

peripheral  and  central,  224 
Deep  sensibility,  58,  294 

tests  of,  63 
Defect  states,  classification  of,  759 

etiology  of,  759 
Defectiveness,  meaning  of  term,  758 
Defectives,  amaurotic  family  types,  762 
cretinism,  764 
epileptic  types,  771 
hydrocephalic  types,  767 
idiot-savants,  774 
inflammatory  types,  773 
microcephalic  types,  767 
mild  grades,  775 
mongolism,  766 
paralytic  types,  771 
sclerotic  types,  763 
sensorial  types,  773 
syphilitic  types,  773 
traumatic  types,  771 
Delire  de  toucher,  612 
Dehrium  acutum,  713 
collapse,  715 
fever,  713 
grave,  715 
senile,  748 
tremens,  722 
Dejerine-Sottas  type  of  muscular  atro- 
phy, 327 
Dementia  and  idiocy,  758 
paranoides,  698 

precox,  ambivalency  of  ideas,  688 
"April  weather"  behavior,  686 
archaic  reactions,  688,  700 
attention,  failure  of,  686 
autistic  thinking,  685 
biological  reactions,  684 
catalepsy,  696 
catatonia,  694 

physical  symptoms,  697 
catatonic  excitement,  695,  696 
rigidity,  688 
stupor,  695 
type  of  reaction,  703 
command  automatism,  696 
complex  indicators,  688 
compromise  formations,  704 
concept  of,  682 
conflict  in,  701 
convulsive  seizures,  690 


Dementia  precox,  course  and  progress, 

705 
delusional  systems,  698 
delusions,  687 
dementia  simplex,  690 
diagnosis,  706 
displacement,     mechanism    of, 

686  _ 
echolaha,  696 
echopraxia,  696 
emotional  deterioration,  692 

dulness,  685 
etiology,  683 
exciting  causes,  684 
fetal  attitude,  702 
formes  frustes,  691 
forms,  690 

formulation  of  disease,  700 
general  considerations,  682 
hebephrenia,  692 
hebephrenic  type   of  reaction, 

703 
heredity  in,  683 
history  of,  683 
interest,  lack  of,  686 
intrapsychic  ataxia,  685 
mannerisms,  688 
memory,  disorders  of,  686 
Meyer's  concept  of,  684 
mixed  and  atypical  states,  698 
mode  of  onset,  690 
motor  disturbances,  688 
muscular  tension,  696 
natm-e  of,  699 
negativism,  688 
neologisms,  688 

orientation,  disturbances  of,  686 
paranoid  forms,  697 

type  of  reaction,  704 
paraphrenia,  698 
pathology,  699 
poverty  of  ideas,  692 
prophylaxis,  710 
psychoanalysis  in,  709 
saltatory  associations,  703 
sensorium,  disorders  of,  687 
"shut  in"  character  as  factor 

in,  684 
splitting    of    personality,    682, 

685,  700 
stereotypies,  688 
suggestibility,  688 
symbolisms  of  the  conflict,  687 
symptom  activities,  688 
symptoms,  684 
mental,  684 
physical,  690 
thinking,  shallowness  of,  687 
thought  deprivation,  703 
dilapidation  of,  687 
looseness  of  train  of,  692 
toxic  factors  in  cause  of,  684 
treatment  of  mental  conditions, 

708 


INDEX 


785 


Dementia  precox,  treatment  of  physical 
conditions,  708 
social  relations,  709 
unconscious,  outcrop  of,  701 
unpsychological  appearance  of, 

685 
and  vegetative  nervous  system, 

703 
waxy  flexibility,  696 
ways  of  getting  well,  703 
wish-fulfilling    delusional    sys- 
tems, 694 
withdrawal  from  reality,  686 
Depressio  apathetica,  745 
Dermoids  of  brain,  494 
Desires,  594 

frustrated,  595 
Diabetes  in  ancestry,  24 
Diadokokinesis,  tests,  38,  55 
Diaschisis,  479 

Diplococcus  intracellularis,  447 
Diplopia,  tests  for,  32 
Dipsomania,  610,  727 
Displacement,  mechanism  of,  74 
Distortion,  mechanism  of,  74 
Doubts,  609.    See  Compulsion  Neurosis. 
Dream  analysis,  627 
Dreams,  74,  595,  604 
latent  content  of,  74 
manifest  content  of,  74 
Drimkenness,  pathological,  721 
Dualistic  hypothesis,  700 
Dura,  inflammation  of,  444 
Dural  disease,  441 
Dysarthrias,  41,  410 
Dyspituitarism,  161,  163 
Dystonia  musculorum  deformans,  431 
Dystrophia  adiposogenitalis,  162 
Dystrophies,   muscular,  328.     See  Mus- 
cular dystrophies. 


Ebbinghau's  test,  71 
Education,  26 
Egocentricity,  597 
Emancipation,  597 
Embohsm,  cerebral,  470 
Encephalitis,  acute,  diagnosis  of,  461 
pyogenic  types,  460 
Strtimpell's,  of  children,  460 
symptoms,  459 
therapy,  461 
etiology,  459 
historical,  458 
influenza,  459 
subcortical,  752 
Enchondromata  of  brain,  494 
Endocrinopathies,  132 
Endotheliomata,  494 
Enem-esis,  26 
Environment,  594 
^Epicritic  sensibility,  58,  294 


Epicritic  sensibility,  tests  of,  59 
Epilepsies,  affect  in,  670,  675 

alcoholic,  727 

in  ancestry,  24 

anomalous   and   borderland    condi- 
tions, 670 

the  aura,  673 

of  Bratz  and  Leubuscher,  675 

classical,  heredity  in,  671 

colony  treatment  of,  681 

continuous,  678 

diagnosis  of,  678 

early,  670 

general  considerations,  667 

genuine,  669 

grand  mal,  673 

of  gross  brain  disease,  670 

idiopathic,  669 

isolated  seizures,  678 

Jacksonian  type,  677 

late,  670 

levels  of  discharge,  669 

meaning  of  attack,  675 

myoclonus,  678 

nocturnal,  678 

paranoid  states  in,  675 

pathological  groups,  670 

and   period   of   wish-fulfillment  by 
incoordinate  movements,  676 

petit  mal,  674 

as  problem  of  energy  distribution, 
667 

psychic,  674 

and  psychoanalysis,  681 

the  seizure,  673 

serial  attacks,  678 

status  epilepticus,  678 

symptom  groups,  670 

symptomatic,  681 

of  toxic  and  infectious  origin,  670 

transitory  conditions  in,  674 
states  of  ill  humor  in,  675 

treatment  of  the  attack,  679 
between  attacks,  680 
of  status,  679 

varieties  of  convulsive  seizures,  678 
Epileptic  automatism,  674 

confusion,  674 

constitution,  672 

delirium,  674 

dementia,  673 

depression,  674 

dipsomania,  675 

dream  states,  674 

ectasy,  675 

equivalent,  674 

excitement,  674 

fugues,  675 

furor,  674 

stupor,  674 
EpUeptics,  polyvalent  sexuality  of,  672 

social  position  of,  681 
Equihbration  tests,  38 
Erb's  palsy,  288 


50 


786 


INDEX 


Erb's  type  muscular  dystrophy,  333 
Erb-Westphal's  sign,  51 
Erb-Zimmerlin  type  muscular  dystrophy, 

319 
Erysipelas,  717 
Erythromelalgia,  101 
Esophagus,  vegetative  innervation  of,  89 
Examination,  methods  of,  neurological, 
17 
objective,  28 
Exanthems,  717 
Exhaustion  psychoses,  714 
Exhibitionism,  779 
Exophthalmic  goitre,  144 
course,  151 
diagnosis,  150 
etiology,  149 
forms,  150 
occuirence,  151 
pathology,  149 
symptoms,  144 
treatment,  151 
Explosive  diathesis  of  Kaplan,  733 
External  cutaneous  nerve,  302 

popliteal  nerve,  302 
Extremities,  lower,  examination  of,  48 

upper,  examination  of,  44 
Eye  palsies,  chronic  progressive,  202 
reflexes,  paths  of  chief,  33 
sympathetic,  85 
Eyeball  apraxias,  209 
Eyes,  methods  of  testing,  30 


Facial  hemiatrophy,  progressive,  215 
lesions,  pontine,  219 
nerve,  215 

diseases  of,  215,  218 

tests  of,  36 
palsies,  cortical,  218 

non-geniculate,  222 

peripheral,  219 
Fatigue  neurosis,  621 
Febrile  psychoses,  712 
Feeble-mindedness,  581.    See  Defectives. 

definition,  760 
Fetichism,  778 
Fever  delirium,  713 

epileptiform  excitement  in,  713 

infectious  dream  state  in,  714 
Fibromata  of  brain,  494 
Finckh  test,  70,  71 
Flexner  serum,  451,  452 
Folie  de  doute,  617 
Forced  movements,  410 
Fore-conscious,  596 
Four  reactions,  518 
Foville's  syndrome,  197,  198,  362 
Franz's  esthesiometer,  59 
Friedmann's  complex,  733 
Friedreich's  ataxia,  414 
FulfiUment,  597 


G 


Gangrene,  multiple  neurotic,  of  skin, 

130 
Gastro-intestinal  ferments,  92 
Geniculate  ganglion,  inflammation  of,  222 

syndrome,  220 
Genital  mechanisms,  95,  96,  97 

syndromes,  167 
Genito-urinary  system,  innervation  of,  94 
Gigantism,  161 
Gliomata  of  brain,  493 
Glossopharyngeal  nerve,  tests  of,  44 
Goitre,  140 

exophthalmic,  144 

course,  151 

diagnosis,  150 

etiology,  149 

forms,  150 

occmrence,  151 

pathology,  149 

symptoms,  144 

treatment,  151 
heart,  140 
Gonads,  167,  169 
Gonorrhea,  27 

Gower's  type  muscular  dystrophy,  335 
Grippe,  717 


H 


Habits,  27 

sexual,  27 
Hallucinosis,  alcohoUc,  725 
Headache,  112 
Hearing  scotomata,  224 
Heart  disease,  psychoses  of,  738 

innervation  of,  98 
Heine-Medin's  disease,  197 
Hematomyelia,  344 
Hemorrhage  of  cerebellum,  415 

cerebral,  470 
Hemianesthesia,  474 
Hemianopsia,  30,  193,  487 
Hemiatrophy,  progressive  facial,  215 
Hemiplegia,  474 

hysterical,  489 
Herpes,  222 

zoster,  272.    See  Zoster. 
Heterosexual  stage,  598 
History,  family,  17,  18,  67 

of  patient,  67 

subjective,  28 
Homosexual  stage,  598 
Homosexuahty,  778 
Hormones,  171,  172 
Horner's  syndrome,  86 
Huntington's    chorea,    433,    437.      See 

Chorea,  Huntington's. 
Hunt's  syndrome,  220 
Hydrocephalus,  455,  581 
Hyperalgesia,  cutaneous  reflex  zones  of, 

248,  249 
Hypergenitalism,  167 


INDEX 


787 


Hyperpituitarism,  161 
Hyperthyreosis,  144 
Hypogenitalism,  167 
Hypoglossal  nerve,  anatomy  of,  234 
disorders  of,  236 
tests  of,  44 
Hypophysis,  diseases  of,  161 
Hypopituitarism,  161,  162 
Hypothyreoses,  133 

symptoms  of,  134 

types  of,  135 
Hysteria,  afTects  in,  600 

amnesias  of,  602 

anxiety,  612 

complexes,  599 

conversion,  600 

crises,  602 

dissociation,  599 

doubling  of  personality,  599 

episodic  phenomena,  602 

historical,  598 

infantilism  of,  603 

interparoxysmal  symptoms,  604 

mechanism  of,  599 

motility  disturbances,  601 

phantasies,  604 

repression,  599 

sensibility  disturbances,  601 

speech  disturbances,  601 

split-off  complexes,  599,  603 

sphtting  of  personality,  604 

stigmata,  604 

symptoms,  601 

analysis  of,  602 

visceral  disturbances,  602 
Hysterie  grande,  598 


Idiocy.    See  Defectives. 

definition,  760 

dementia  and,  758 
Idio-imbicility,  definition,  760 
Idiot-savants,  774 
Imbecility.    See  Defectives. 

definition,  760 

moral,  definition,  760 
Individual,  600 
Infection  delirium,  713 

-exhaustion  psychoses,  712 

treatment  of,  717 
Infections,  27 

Insanity  of  double  form.     See  Manic- 
depressive  psychoses. 

meaning  of  term,  758 
Injury,  28 

Intermittent  claudication,  103,  252 
Internal  secretions,  132 
Inter-renal  syndromes,  167 
Intestines,  disorders  of,  91,  92 

innervation  of,  90 
Intoxications,  285 
Introspectiveness,  597 


Introversion,  597 

Involution  melancholia,  delusions,  741 

endopsychic  conflict,  744 

manic-depressive  psychosis  and, 
742 

symptoms,  740 

terminations,  741 

treatment,  744 
period,  742 
Irregular  palsies,  203,  209 


Jacobsohn's  radius  reflex,  49 
Joint  sense,  tests  of,  63 


Kltjmpke  syndrome,  363 
Knee-jerk,  51 
Korsakow's  psychosis,  724 

and  presbyophrenia,  749 
syndrome,  201,  281,  733 

in  brain  tumor,  499,  502,  503, 
505 


Labyrinth,  functions  of,  225 
Labyrinthine  and  cerebellar  disturbances, 

differential  diagnosis  of,  233 
Lacunar  syndromes,  489 
Landouzy-Dejerine    type    of    muscular 

dystrophy,  334 
Landry's  paralysis,  313 
Lange's  colloidal  gold  test,  520 
Larynx,  examination  of,  40 
Lasegue's  test,  64 
Lateral  associated  palsy,  203,  206 
sclerosis,  congenital  types,  353 

group,  351 

hereditary  familial  types,  353 

infantile  types,  353 

mixed  types,  353 

unilateral    ascending    and    de- 
scending types,  353 
Law  of  avalanche,  668 
Lenticular  degeneration,  progressive,  173 
Leptomeningitis,  acute,  causes,  446 

course,  450 

differential  diagnosis,  453 

prognosis,  450 

symptoms,  447 

treatment,  453 
chronic,  454 
Lipomata  of  brain,  494 
Little's  disease,  353 
Liver  syndromes,  173 
Long  thoracic  nerve,  301 
Love,  infantile,  for  family,  596 
Lowi's  reaction,  87,  146 


788 


INDEX 


Luetin  test,  520 

Lymphocytosis  of  cerebrospinal  fluid  in 
syphilis,  517 


M 


Magendie-Hertwig  position,  207 

syndrome,  411 
Malaria,  717 
Mania.    See  Manic-depressive  psychosis. 

acute  delirious,  715 
Manias.     See  Compulsion  neurosis. 
Manic-depressive  psychosis,  acute  mania, 
637 
melancholia,  640 
agitated  depression,  647 
akinetic  mania,  647 
ambivalency  of  ideas,  649 
attention,  disorders  of,  638 
chronic  depression,  642 

mania,  640 
clang  association,  636 
coiu"se,  650 
cyclothymias,  645 
delirious  mania,  639 
delusions,  639 
depression  with  flight  of  ideas, 

647 
depressive  mania,  647 
phase,  640 
stupor,  642 
differential  diagnosis,  650 
difficulty  of  thinking,  640 
distractibUity,  636 
emotional  depression,  640 

exaltation,  634,  639 
etiology,  633 

feeling  of  inadequacy,  641 
flight  of  ideas,  635 
general  concept  of,  632 
hypomania,  634 
involution  melancholia,  648 
leveling  of  ideas,  639 
maniacal  stupor,  647 
manic  phase,  634 
mixed  states,  647 
nature  of,  648 
periodical  types,  642 
pressiire  of  activity,  637 

of  speech,  637 
prognosis,  650 
prophylaxis,  654 
psychomotor      activity,      634, 
639 
retardation,  640 
simple  retardation,  640 
treatment,  651 
unproductive  mania,  647 
Marie's  hereditary  cerebellar  atrophies, 

413 
Masochism,  778 
Masselon  test,  69,  71 
Mastiirbation,  27,  621,  778 


Melanchoha.    See  Manic-depressive  psy- 
chosis. 

involution.     See  Involution  melan- 
cholia. 

vera,  745 
Memory,  tests  of,  69 
Mendel-Bechterew  reflex,  54 
Meniere-like  attacks,  229 

syndrome,  230 
Meningeal  apoplexy,  441 

hemorrhage,  traumatic,  441 
Meninges,  diseases  of,  441 
Meningitis,  cerebrospinal,  causes,  446 
epidemic,  450 
symptoms,  447 

course,  450 

cranial  nerve  signs;  448 

differential  diagnosis,  453 

headache  in,  447 

hyperesthesia  in,  447 

infectious,  450 

irregular  symptoms,  449 

lumbar  puncture,  449 

mental  symptoms,  447 

motor  irritation,  448 

nausea  and  vomiting,  447 

paralytic  phenomena,  448 

prognosis,  450 

reflexes,  449 

serous,  453 

stiffness  of  neck,  447 

syphilitic,  of  base,  524 
of  convexity,  526 

temperature,  448 

treatment,  453 

tuberculous,  452 
Meningococcus  intracellularis,  450 
Menstrual  history,  28 
Mental  disorder  in  ancestry,  23 

examination,  69 
methods,  66 

and  physical,  relation  of,  701 
Metamere,  type  of,  80 
Midbrain,  diseases  of,  385 

syndrome,  385 
Migraine  in  ancestry,  24 

classical,  107 

diagnosis,  117 

ophthalmic,  104 

ophthalmoplegic,  121 

psychical  disturbances,  115 

scotomata,  110 

secretory  disturbances,  115 

symptomatic,  116 

symptoms,  motor,  111 
sensory,  108 

treatment,  117 

trophic  disturbances,  115 

vasomotor  disturbances,  114 

visual  phenomena,  109 
MUiary  plaques  in  senile  dementia,  750 
Millard-Gubler  syndrome,  197,  397,  420 
Millard-Gubler-Foville  syndrome,  398 
Miscarriages,  28 


INDEX 


789 


Mobius'  symptom,  207 

Mongolism  and  cretinism,  705 

Motor  neurones,  central,  of  oculomotor 
nerves,  203 

Motor-oculi  nerve,  tests  of,  31 

Mucous  and  salivary  glands,  vegatative 
control  of,  87 

Miiller's  muscle,  85 

Multiple     sclerosis,    apoplectic   attacks, 
379 
ataxia,  376 

bladder  involvement,  379 
diagnosis,  381 

eighth  nerve  symptoms,  375 
epileptiform  attacks,  379 
etiology,  371 
eye  muscle  palsies,  374 
fifth  nerve  symptoms,  375 
forms,  characteristic,  380 

non-characteristic,  381 
gait  disturbances,  377 
historical,  368 
intention  tremor,  376 
involuntary  laughing  and  cry- 
ing, 379 
lumbar  puncture,  379 
motor  disturbances,  376 

power,  377 
muscular  atrophies,  377 
ocular  symptoms,  374 
olfactory  symptoms,  372 
optic  symptoms,  372 
pathogenesis,  382 
pathology,  382 
prognosis,  383 
psychoses  of,  737 
sensory  phenomena,  377 
seventh  nerve  symptoms,  375 
signs,  other,  378 
skin  reflexes,  378 
spastic  paresis,  377 
speech  disturbances,  375 
symptoms,  372 
taste  modifications,  376 
tendon  reflexes,  378 
tenth  nerve  symptoms,  376 
therapy,  383 
trophic  signs,  378 
vasomotor  signs,  378 
writing,  377 

Muscle  sense,  tests  of,  63 
syndromes,  174 

Muscles  of  lower  extremities,  examina- 
tion of,  48 
of  trunk,  examination  of,  48 
of  upper  extremities,  examination  of, 
44 

Muscular  atrophies.    (See  Muscular  dys- 
trophies. 
Aran-Duchenne  type,  320,  321 
bulbopontine  types,  323 
Charcot-Marie-Tooth  type,  325 
Dejerine-Sottas  type,  327 
infantile  hereditary  forms,  323 


Muscular  atrophies,  neural,  neuritic,  or 
spinal  neuritis,  325 
peroneal-forearm  type,  325 
pontomesencephalic  forms,  324 
primary  progressive,  319 

classification,  319 
progressive,  321 
nuclear,  320 
tabetic  type,  327 
Werdnig-Hoffmann  form,  320, 
323 
dystrophies,     328.      See    Muscular 
atrophies, 
amyotonia  congenita  type,  334 
distal  type,  335 
Duchenne  type,  331 
Erb  type,  333 
Erb-Zimmerlin  type,  319 
facio-scapulo-humoral  type,  334 
forms,  330 
Gower's  type,  335 
juvenile  form,  333 
Landouzy-Dejerine  type,  334 
mixed  forms,  319 
myatonia  atrophica  type,  336 
pseudohypertrophic  type,  331 
Musculospiral  nerve,  302 
Myasthenia  gravis,  174 
Myatonia  atrophica,  336 
Myopathies,  328 
Myotonia  congenita,  176 
Myxedema,  133 

congenital  forms,  136 

diagnosis,  136 

idiopathic,  135 

operative,  135 

prognosis,  136.    See  Cretinism. 

therapy,  136.    See  Cretinism. 


N 

Nail  biting,  26 
Narcissism,  778 
Narcotics,  27 
Neck  sympathetic,  88 
Necrophilia,  779 
Neosalvarsan,  588 
Neuralgias,  245 

brachial,  260 

cervico-occipital,  259 

of  coccygeal  plexus,  272 

com'se,  250 

crural,  270 

definition,  245 

diagnosis,  251 

diaphragmatic,  260 

etiology,  246 

femoral,  271 

idiopathic,  250 

intei  costal,  263 

localized  forms  of,  255 

lumbar  plexus,  264 

lumbo-abdominal,  270 


790 


INDEX 


Neuralgias,  obturator,  271 

painful  points  in,  261 

prognosis,  253 

pudendal  plexus,  271 

reflex,  246,  252 

symptoms,  247 

testicular,  270 

treatment,  253 

trigeminal,  255 
Neurasthenia,  620 
Neurasthenic  pains,  252 
Neuritis,  252,  278  _ 

alcoholic  multiple,  281 

arsenical,  284 

diphtheritic,  285 

infectious  disease  types,  285 

influenza,  285 

intoxication,  285 

lead,  283 

multiple,  279 

optic,  axial,  acute,  186 
chronic,  187 
hereditary  forms,  189 
diffuse,  190 

interstitial  peripheral,  189 
Nem'ofibromata  of  brain,  494 
Neurological  examination,  68 
Neuroses,  593 

actual,  613 

mixed,  623 

treatment  of,  623 
Nerve  of  Bell,  301 

external  cutaneous,  302 
popliteal,  302 

long  thoracic,  301 

musculospiral,  302 
Nerve  trunks,  tests  of  sensibility  of,  63 
Nervous  disorder  in  ancestry,  24 
Nonne's  findings  in  syphilis,  519 
Nucleus  of  personality,  754,  756 
Nystagmus,  229,  408 

central,  203,  209 

supplementary  tests  in  presence  of, 
31 


Obsessions.     See  Anxiety  neurosis  and 

Compulsion  neurosis. 
OccipitocoUaris  herpes,  222 
Oculogyric  fibers,  198 
Oculomotor  nerve  palsies,  197 
nerves,  diseases  of,  197 
palsies,  irregular,  203,  209 

lateral  associated,  203,  206 
vertical  associated,  203,  206 
Olfactory  nerve,  179 
tests  of,  29 
tract,  diseases  of,  179 
Olivopontocerebellar  atrophy,  413 
Ophthalmic  migraine,  104 

psychosis,  735 
Ophthalmoplegia,    chronic    progressive, 
324 


Ophthalmoplegia  externa,  34 

interna,  34 
Ophthalmoplegic  migraine,  121 
Opiumism,  727 
Oppenheim  reflex,  54 

type  of  muscular  dystrophy,  334 
Optic  atrophy,  188,  190 
nerve,  183,  186 
tests  of,  29 
pathways,  diseases  of,  183 
tract,  disease  of  cortex  in  lesions  of, 
195 
of  thalamus  in  lesions  of, 
194 
Osteomata  of  brain,  494 
Overdetermination,  75 


Pachymeningitis  externa,  444 
hypertrophic  cervical,  577 
interna  hemorrhagica,  444 
simplex,  444 
Pain  tests,  61 
Palmos,  423 

Palsies,  brachial  plexus,  286 
total,  287 
bulbar,  chronic  progressive,  323 
lumbosacral,  291 
mixed  types,  289 
periodic,  121 
peripheral,  292 

traumatic,  293 
plexus,  286 
sacral,  292 
Palsy,  Erb's,  288 

inferior  root  type,  288 
lead,  283 

superior  brachial  plexus,  288 
Pancreas,  170 
Pancreatic  syndrome,  172 
PapilHtis,  187 

PapiUomacular  bundle,  188 
course  of,  189 
scheme  of,  186 
Paracusis,  224 

Paradoxical  reflex  of  Gordon,  54 
Paralysis  agitans,  contractions,  425 
course  and  progress,  429 
diagnosis,  663 

differential,  429 
equilibrium  disturbances,  424 
etiology,  424 
history,  423 
hypertonus,  425 
mask,  425 

muscular  weakness,  426 
ocular  palsies,  426 
pathology,  430 
propulsion,  427 
psychic  disturbances,  428 
psychoses  of,  737 
reflexes,  428 


INDEX 


791 


Paralysis  agitans,  rigidity,  425 

secretory  disturbances,  428 

sensorimotor  disturbances,  425 

symptoms,  424 
sensory,  428 

therapy,  430 

treatment,  664 

tremor,  427 

trophic  distm'bances,  428 

vasomotor  disturbances,  428 
of  convergence,  203,  207 
Paranoia,  acquired,  658 
acute,  660 
alcoholism  and,  660 
combinatoria,  658 
delirium  of  interpretation,  659 

of  revindication,  659 
delusions,  661 

dementia  precox  and,  660,  661 
description  of,  657 
erotic,  658 
expansive,  658 
Freud's  conception  of,  662 
general  concept  of,  655 
hallucinatoria,  658 
interpretation,  660 
inventive,  658 
of  Kraepelin,  659 
of  Krafft-Ebing,  658 
late,  658 
litigious,  658 
of  Magnan,  657 

manic-depressive  psychosis  and,  661 
original,  658 
paraphrenia  confabulans,  659 

expansiva,  659 

phantastica,  659 

systematica,  659 
persecutory,  658 
phantastic,  700 
projection,  mechanism  of,  662 
psychoanalysis  and,  665 
querulous,  658 
reformatory,  658 
rehgious,  658 

of  Serieux  and  Capgras,  658 
suspiciousness,  affect  of,  661 
Paraphrenia,  659.    See  Paranoia. 
Parasympathetic  nervous  system,  79 
Paiathyreopriva,  158 
Parathyroid  synchome,  152 
Parathyroids,  169 
Paresis,  agitated  forms,  547 

cerebrospinal  fluid  findings  in,  541 
character  alterations,  539 
convulsive  phenomena,  540 
defects  of  retention  and  memory, 

537 
delusions,  538 
dementing  forms,  543 
depressed  forms,  543 
emotional  activity  alterations,  538 
expansive  types,  544 
eye  symptoms,  540 


Paresis,  forms,  542 

impoverishment  of  ideas,  538 

irregular  types,  548 

juvenile,  549 

Lissauer's  type,  548 

motor  incoordinations,  540 

neurological  signs,  539 

reflexes,  alterations  of,  541 

remissions,  542 

sensibiUty,   changes    in,   cutaneous, 
539 

speech  disorders  of,  540 

symptoms,  535 

syphihtic  etiology,  533 

taboparesis,  548 

trophic  disturbances  in,  542 

types  of,  536 

vasomotor  disturbances,  542 
Patellar  tendon  reflex,  51 
Peduncular  pathways,  symptoms  due  to 
lesions  of,  390,  392,  394,  396 

syndromes,  385 
anterior,  388 
posterior,  386 
Pellagra,  psychoses  of,  738 
Pelz-atypical  Thomsen's  disease,  336 
Periodic  palsies,  121 

Peripheral  nerves,   injm-ies  to  terminal 
branches  of,  300 
sensory  disturbances  of,  245 
Perivascular  gliosis,  753 
Phantasies,  597 

conscious,  604 
Pharmacological  tests  of  vegetative  ner- 
vous system,  83 
Pharynx,  examination  of,  39 
Phobias.  See  Anxiety  neurosis  and  Com- 
pulsion neurosis. 
Physical  examination,  68 
Physico-chemical  systems,  79 
Pia,  diseases  of,  446 
Pilomotor  system,  131 
Pineal  syndrome,  165,  197 
Pituitary,  169 

diseases  of,  161 
Pneumonia,  718 

Pohoencephahtis,     acute     hemorrhagic, 
725 

hemorrhagic  superior,  460 

superior,  197 
Polioencephalomyelitis,  305,  460 

abortive  forms,  315 

acute,  305 

ascending  form,  313 

ataxic  forms,  314 

atrophic  stages,  312 

bulbar  and  pontine  forms,  313 

diagnosis,  315 

encephalic  or  cerebral  form,  314 

meningitic  forms,  314 

nervous  symptoms  of,  309 

pathology,  306,  307,  308 

polyneuritic  forms,  315 

prodromata,  309 


792 


INDEX 


Polioencephalomyelitis,  prognosis,  316 

prophylaxis,  317 

sensory  disturbances,  312 

spinal  symptoms,  310 

symptoms,  309 

treatment,  317 

Wickmann's  types,  310 
Poliomyelitis.      See   Polioencephalomye- 
litis. 

acute  posterior,  272 

chronic,  320 
Polyglandular  syndromes,  165,  172 
Polyneuritis,  279.    See  Korsakow's  psy- 
chosis. 
Pons,  diseases  of,  385  • 
Pontine  facial  lesions,  219 

syndromes,  212,  385,  398 
anterior,  397 

cephalic,  389 

and  posterior  internal,  391 
middle,  393,  412 
posterior,  199,  362,  395 
Popliteal  nerve,  302 
Porencephalus,  771 
Postfebrile  psychoses,  714 
Presbyophrenia,  748 

formes  frustes,  750 

Korsakow's  psychosis  and,  749 
Presenile  psychoses,  740 
delusional,  744 
Prison  psychoses,  777 
Protopathic  sensibility,  58,  294 

tests  of,  61 
Psammomata  of  brain,  494 
Pseudologia  phantastica,  777 
Pseudoparesis,  alcoholic,  726 

syphilitic,  552 
Pseudotabes,  569 
Pseudotabetic  syndromes,  558 
Psychical  systems,  593 
Psychoanalysis,  72,  623 

technique  of,  75,  624 
Psychological  age,  760 

classification,  761 

development,  594 

facts,  627 
Psychoneuroses,  593,  598 
Psychopathic  constitution,  777 
Psychoses  associated  with   organic  dis- 
eases, 732 
Ptosis,  34 
Pupils,  tests  of,  32 
Pyramidal  tract,  56 


Questionnaire,  17 


Radicular  segment  distribution,  276 
Radiculitis,  274 


Radiculitis,  brachial,  277 

cervical,  277 

syphilitic,   sensory    disturbances  in, 
275 
Radiculoganglionic  syndrome,  272 
Raynaud's  disease,  102 
Rectum,  disorders  of  functions  of,  93,  94 

innervation  of,  92 
Reflexes,  68 

of  lower  extremities,  51 

of  trunk,  49 

of  upper  extremities,  49 
Reproduction,  instinct  of,  96 
Respiratory  apparatus,   innervation  of, 
97 

mechanisms,  97,  98 
Restiform  body,  400 
Retinitis,  183 

albuminuric,  185 

hemorrhagic,  185 

pigmentosa,  186 

simple,  185 

syphilitic,  185 
Rheumatism,  718 
Rinne  test,  37 
Romberg  test,  38 
Root  segment  distribution,  276 
Rubrospinal  syndrome,  201 


Sadism,  778 

SaUvary  and  mucous  glands,  vegetative 

control  of,  87 
Salvarsan,  588 
Sarcomata  of  brain,  494 
Scanning  speech,  41 
Schemata,  481 

Schizophrenia.    See  Dementia  precox. 
Sciaticas,  264 

Brissaud's  spasmodic,  268 

coiu"se  of,  268 

diagnosis  of,  268 

electrotherapy  of,  270 

etiology  of,  264 

forms,  268 

Fournier's  gonorrheal,  268 

Quenu's  varicose,  268 

symptoms,  265 

therapy,  269 
Scotomata,  30,  187,  188 

in  migraine,  110 
Seasickness,  treatment  of,  231 
Secondary  elaboration,  75 
Secretions,  internal,  132 
Senile  cortical  devastation,  753 

delirium,  748 

deterioration,  simple,  748 

involution,  affectivity  in,  747 
normal,  746 

psychoses,  746 

pathology,  750 
presbyophrenia,  748 


INDEX 


793 


Senile  psychoses,  treatment,  750 
Sensation,  loss  of,  64 
Sensations,  spontaneous,  64 
Sensori-motor  neurology,  179 

systems,  179 
Sensory  fibers,  three  systems  of,  294 

impulses,  pathways  from  thalamus 

to  cortex,  481 
nerve  division,  incomplete,  295 
stages  of  recovery,  296 
nerves,  peripheral,  disturbances  of, 

245 
nervous  system,  examination  of,  55 
paths,  385 

symptoms    of    interruption    in 
brain  stem,  387 
Sensibility,  deep,  294 
epicritic,  294 
protopathic,  294 
scheme  for  testing,  64 
Seventh  nerve,  lesions  at  pons,  222 
Sexual   instinct,    qualitative   anomalies, 
778 
quantitative  anomalies,  778 
maturity,  607 
organs,  innervation  of,  95 
Shingles,  272 
Shocks,  27 

"Shut  in"  character,  684 
Skew  deviation,  209,  411,  418 
Smell,  tests  for,  29 
Spastic  anemias, .  102 

Speech  disturbances,  237,  238,  239,  240 
clinical  forms  of,  240 
examination  of,  41 
tests  of,  69 
Spinal  accessory  nerve,  41,  300 
anatomy  of,  233 
disturbances  of,  234 
cord,  afferent  impulses,  grouping  of, 
344,  345 
compression  of,  348 

by  bony  hypertrophies,  348 
by  caries,  348 
cross  section,  effect  of,  on  long 

fiber  tracts,  341 
fracture    and    dislocation    syn- 
dromes, 341 
diagnosis,  345 
prognosis,  346 
symptoms,  342 
therapy,  348 
intramedullary  lesions,  344 
lesions  of,  303 
localizations,  304 

at  different  levels,  343 
syndromes,  303 

transverse  lesion,  complete,  342 
tumors,  349 
Stammering,  26 
Status  corporis,  65 

thymolymphaticus,  167 
Stellwag's  sign,  145 


Stereognosis,  64 

Stomach,  disorders  of,  90,  91,  92 

innervation  of,  90 
Stones,  69,  70,  71 
Strlimpell  reflex,  54 
Stuttering,  41 
Suicide,  653 
Suprarenal,  167,  169 
Suprathalamic  pathways,  478 
Sweat  secretory  mechanisms,  130 
Sydenham's  chorea,  433,  434 
Symbolic  systems,  593 
Symbolism",  75,  597,  610 
Sympathetic  and  autonomic  nervous  sys- 
tems, comparison  of  antagonistic 
actions  of,  84 
division  of  vegetative  nervous  sys- 
tem, 82 
nervous  system,  79 
Sympathicotonic  products  of  endocrin- 
ous system,  83 
Symptoms,  meaning  of,  629 
Syphilis,  511 

in  ancestry,  24 
blood  tests,  514 
cerebral,  dizziness  in,  531 
headache  in,  531 
insomnia  in,  531 
local  symptoms,  532 
parenchymatous,      532.        See 

Paresis, 
psychical  disturbances,  531 
sleepiness  in,  531 
vascular   types,   symptoms  of, 
529         ■ 
cerebrospinal  fluid,  changes  in,  516 
chemical    examination    of, 

517 
cytology  of  515 
lymphocytosis,  517 
tests,  514 
clinical  forms,  522 
congenital,  577,  580 
of  cranial  bones,  523 
diagnosis,  514 
eye  reflexes,  520 
feeble-mindedness  in,  581 
hereditary,  577 

effects  of,  577 
history,  512 
hydrocephalus,  581 
hypertrophic     cervical     pachymen- 
ingitis, 577 
of    nervous    system,  treatment    of, 

583 
Nonne's  findings  in  519 
organism,  514 

treatment  by  injection,  585 
by  inunction,  584 
by  iodides,  591 
by  mercary  by  mouth,  591 
by  methods  of  Swift  and  Ellis, 

590 
by  neosalvarsan,  588 


794 


INDEX 


Syphilis,    treatment  by  oleate  of    mer- 
cury, 684 
by  salvarsan,  588 
by  unguentum  hydrargyri,  585 
Syphilitic  meningitis  of  base,  524 
of  convexity,  526 
meningomyelitis,  573 

meningeal  syndromes,  575 
myelitic  syndromes,  575 
root  syndromes,  575 
symptoms,  574 
system  syndromes,  576 
neurasthenia,  552 
psychoses,  550 

cardiac  disease,  associated  with, 

554 
epileptic  forms,  554 
forms  of,  552 
hallucinatory    confused   states, 

554 
hereditary,  554 

manic-depressive  psychosis,  re- 
sembling, 554 
paranoid  forms  with  tabes,  552 

without  tabes,  553 
pseudoparesis,  552 
psychic  trauma,  due  to,  554 
simple  weakness  of  mind,  552 
Syphilomata  of  brain,  495 
Syringobulbia,  364 

Syringomyelia,  bulbar  symptoms,  363 
types,  364 
cervical  type,  365 
course,  366 

and  progress,  364 
differential  diagnosis,  366 
dorsolumbar  types,  365 
etiology,  356 
groupings   of   types,    characteristic, 

365 
historical,  355 
mental  symptoms,  364 
muscular     atrophies     and     trophic 

changes,  359 
pathogeny,  367 
pathology,  366 
reflexes,  361 
sacrolumbar  types,  365 
sensory  dissociation,  356,  358 
symptoms,  356 
therapy,  368 
trophic  disturbances,  361 
visceral  symptoms,  363 


Tabes,  accessory  involvement,  565 
acoustic  nerve,  565 
arthropathies,  565 
ataxia,  562 
blood  serum,  568 
cerebrospinal  fluid,  568 
course  and  variation,  568 


Tabes,  cranial  nerves,  564 

crises,  560 

diagnosis,  569 

etiology,  558 

facial  palsy,  565 

forms,  568 

gait.,  563 

histopathology,  571 

history,  554 

juvenile,  569 

late,  569 

muscular  atrophies,  567 

Nageotte's    scheme    of   location    of 
lesions,  572 

neuralgic  pains,  252 

occurrence,  559 

ocular  palsies,  564 

optic  nerve,  564 

pains,  560 

pathogenesis,  573 

pathology,  570 

pupillary  reflexes,  564 

rapid,  569 

Romberg's  sign,  563 

sensory  involvement,  561 

slow  cases,  569 

stationary  or  benign  cases,  569 

sympathetic    system    in  pathology 
of,  573 

symptoms,  559 
trophic,  565 
visceral,  565 

tendon  reflexes,  563 

trigeminus  involvement,  565 

vagus  involvement,  565 
Taboparesis,  548 
Taste,  examination  of,  40 

fibers,  pathways  of,  235 
Tay-Sachs  disease,  762 
Tear  glands,  sympathetic  innervation  of, 

87 
Teratomata  of  brain,  494 
Tetany,  152,  159  . 

course,  157 

diagnosis,  158 

etiology,  153 

incidence,  153 

pathology,  154 

prognosis,  158 

strumipriva,  158 

symptoms,  155 
Thalamic  syndrome,  474,  476 

affective  reactions  in,  476 
partial,  478 

sensibility  disturbances  in,  477 
Thalamocortical  pathways,  478 
Thalamus  and   cortex,   functional  rela- 
tions, 482 

disease  of,  in  lesions  of  optic  tract, 
194 

relations  of,  in  sensory  paths,  475 
Thermal  sensibility,  epicritic,  60 
Thomsen's  disease,  176 
Thoracic  nerve,  long,  301 


INDEX 


795 


Thrombosis,  cerebral,  470 
Thumb-sucking,  26 
Thymus,  167,  169 
Thyreopathies,  133 
Thyreoses,  133 
Thyroid,  169,  170 
Tic  douloureux,  213,  256 

diagnosis  of,  257 

treatment  of,  258 
Tics,  55 

Tongue  palsies,  236 
Tonic  hyperemias,  101 
Touch,  light,  59 
Toxemias,  occupation,  27 
Toxic  psychoses,  719 

bromides,  728 

carbon  monoxide,  729 

cocain,  728 

diabetes  mellitus,  730 

gastro-intestinal  diseases,  730 

general  considerations,  729 

lead,  729 

mercury,  729 

miscellaneous  intoxicants,  728 

opium,  727 

uremia,  730 
Transfer,  629 
Traumatic  psychoses,  733 

defect  conditions,  735 

insolation,  735 

ophthalmic,  735 

postoperative,  735 

post-traumatic  constitution,  735 
deliria,  734 
Tremor  coactus,  427 
Tremores  paralytoidei,  423 
Tremors,  54 

Trigeminal  herpes  zoster,  213 
nerve,  disease  of,  209 

motor  part,  209 

diseases  of,  210 

sensory  part,  211 

diseases  of,  213 
nem-algia,  255 
neuritis,  213  ■ 
Trigeminus  nerve,  tests  of  motor  part,  34 

of  sensory  portion,  35 
Trochlearis,  197 

nerve,  tests  of,  31 
palsy,  202 
Trophic  disturbances,  64 
Trousseau's  points,  257 
Tuberculomata  of  brain,  495 
Tuberculosis  in  ancestry,  24 
Tuinors  of  brain,  actinomycosis,  495 

anemismal,  495 

carcinomata,  494 

cardiac  signs,  498 

central  convolutions,  503 

chloromata,  494 

chordomata,  494 

corpus  callosum,  507 

diagnosis,  509 

dizziness  in,  498 


Tumors  of  brain,  endotheliomata,  494 

etiology,  493 

fibromata,  494 

focal  symptoms,  500 

frontal  lobe,  501 

general  symptoms,  496 

gliomata,  493 

headache  in,  497 

infectious,  495 

Korsakow's  syndrome  in,  499, 
502,  503,  505 

mental  symptoms,  499 

metaboHc  disturbances,  499 

motor  phenomena,  500 

nausea  in,  498 

occipital  lobes,  506 

optic  nerve  changes,  500 
thalamus,  508 

parasitic  cystic,  495 

parietal  lobes,  504 

prognosis,  509 

respiratory  sign,  498 

sarcomata,  494 

syphilomata,  495 

temporal  lobes,  505 

treatment,  509 

true,  493 

tuberculomata,  495 

varieties,  493 

vomiting  in,  498 
of  cerebellopontine  angle,  421 
of  cerebellum,  416 
of  fourth  ventricle,  420 
of  spinal  cord,  349 
Typhoid  fever,  27,  717 


Ulnar  nerve,  301 
Unconscious,  596 


Vagoparalytic  drugs,  83 
Vagospastic  drugs,  83 
Vagotonic  products  of  endocrinous  sys- 
tem, 83 
Vagus  nerve,  41,  44,  89 
Valleix's  points,  247 

in  tic  douloureux,  256 
Van  Frey's  hairs,  59 
Vascular  apparatus,  98 
Vasomotor  disturbance,  64 
irritability  group,  123 
neuroses,  100,  101 

spastic  anemias,  102 
tonic  hyperemias,  101 
vasomotor  irritability,  123 
Vegetative  nervous  system,  79 

neurology,  79 
Vertical  associated  palsy,  203,  206 
Vertigo,  38,  408 


796 


INDEX 


Vertigoes,  cerebellar,  230 

treatment  of,  230 

vestibular,  228 
Vessels,  abdominal,  vegetative  control  of, 

92 
Vestibular  nerve,  anatomy  of,  226,  227 
cerebral  paths  of,  231 
divisions  of,  229 

pathways,  diseases  of,  222 

vertigoes,  228 
Visceral  neurology,  79 
Vision,  color,  30 

field  of,  ways  of  testing,  31 
Visual  field,  190,  191,  192 
Von  Graefe's  sign,  145,  207 


W 

Wassermann  reaction,  514,  518 

provocative,  520 
Wahnsinn,  depressive,  744 


Weber's  syndrome,  388 

test,  37 
Weber-Gubler  syndrome,  201,  420 
Werdnig-Hoffmann    type    of    muscular 

atrophy,  320,  323 
Wernicke's  hemiopic  phenomenon,  33 

polioencephalitis  superior,  197,  460 
Witzelzucht,  464,  501 
Word-deafness,  243 
Word-dumbness,  243 
Worms,  intestinal,  26 
Writer's  cramp,  244 
Writing  disturbances,  244 


Ziehen  test,  69,  71 

Zona,  272 

Zoster,  ophthalmic,  274 
symptomatic,  273 
zone  of  geniculate,  222 


im 


MBj^ 


.-£. 


l^* 


^^14 


mf^ 


8  0  19% 
TOTT 


1996 


;^=:;r^ 


Im&mSSSi^s 


0C37578120 


^e.-5A^v 


/V 


r' 


\\. 


SS' 


~A- 


^b:^ 


^>^/7, 


'y 


